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class="vector-toc-numb">2</span> <span>Genetics</span> </div> </a> <ul id="toc-Genetics-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Pathophysiology" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Pathophysiology"> <div class="vector-toc-text"> <span class="vector-toc-numb">3</span> <span>Pathophysiology</span> </div> </a> <ul id="toc-Pathophysiology-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Diagnosis" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Diagnosis"> <div class="vector-toc-text"> <span class="vector-toc-numb">4</span> <span>Diagnosis</span> </div> </a> <button aria-controls="toc-Diagnosis-sublist" class="cdx-button cdx-button--weight-quiet cdx-button--icon-only vector-toc-toggle"> <span class="vector-icon mw-ui-icon-wikimedia-expand"></span> <span>Toggle Diagnosis subsection</span> </button> <ul id="toc-Diagnosis-sublist" class="vector-toc-list"> <li id="toc-Types" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Types"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.1</span> <span>Types</span> </div> </a> <ul id="toc-Types-sublist" class="vector-toc-list"> <li id="toc-Type_1" class="vector-toc-list-item vector-toc-level-3"> <a class="vector-toc-link" href="#Type_1"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.1.1</span> <span>Type 1</span> </div> </a> <ul id="toc-Type_1-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Type_1C" class="vector-toc-list-item vector-toc-level-3"> <a class="vector-toc-link" href="#Type_1C"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.1.2</span> <span>Type 1C</span> </div> </a> <ul id="toc-Type_1C-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Type_2" class="vector-toc-list-item vector-toc-level-3"> <a class="vector-toc-link" href="#Type_2"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.1.3</span> <span>Type 2</span> </div> </a> <ul id="toc-Type_2-sublist" class="vector-toc-list"> <li id="toc-Type_2A" class="vector-toc-list-item vector-toc-level-4"> <a class="vector-toc-link" href="#Type_2A"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.1.3.1</span> <span>Type 2A</span> </div> </a> <ul id="toc-Type_2A-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Type_2B" class="vector-toc-list-item vector-toc-level-4"> <a class="vector-toc-link" href="#Type_2B"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.1.3.2</span> <span>Type 2B</span> </div> </a> <ul id="toc-Type_2B-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Type_2M" class="vector-toc-list-item vector-toc-level-4"> <a class="vector-toc-link" href="#Type_2M"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.1.3.3</span> <span>Type 2M</span> </div> </a> <ul id="toc-Type_2M-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Type_2N_(Normandy)" class="vector-toc-list-item vector-toc-level-4"> <a class="vector-toc-link" href="#Type_2N_(Normandy)"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.1.3.4</span> <span>Type 2N (Normandy)</span> </div> </a> <ul id="toc-Type_2N_(Normandy)-sublist" class="vector-toc-list"> </ul> </li> </ul> </li> <li id="toc-Type_3" class="vector-toc-list-item vector-toc-level-3"> <a class="vector-toc-link" href="#Type_3"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.1.4</span> <span>Type 3</span> </div> </a> <ul id="toc-Type_3-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Comparison" class="vector-toc-list-item vector-toc-level-3"> <a class="vector-toc-link" href="#Comparison"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.1.5</span> <span>Comparison</span> </div> </a> <ul id="toc-Comparison-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Platelet-type" class="vector-toc-list-item vector-toc-level-3"> <a class="vector-toc-link" href="#Platelet-type"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.1.6</span> <span>Platelet-type</span> </div> </a> <ul id="toc-Platelet-type-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Acquired" class="vector-toc-list-item vector-toc-level-3"> <a class="vector-toc-link" href="#Acquired"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.1.7</span> <span>Acquired</span> </div> </a> <ul id="toc-Acquired-sublist" class="vector-toc-list"> </ul> </li> </ul> </li> </ul> </li> <li id="toc-Treatment" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Treatment"> <div class="vector-toc-text"> <span class="vector-toc-numb">5</span> <span>Treatment</span> </div> </a> <ul id="toc-Treatment-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Epidemiology" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Epidemiology"> <div class="vector-toc-text"> <span class="vector-toc-numb">6</span> <span>Epidemiology</span> </div> </a> <ul id="toc-Epidemiology-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-History" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#History"> <div class="vector-toc-text"> <span class="vector-toc-numb">7</span> <span>History</span> </div> </a> <ul id="toc-History-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Other_animals" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Other_animals"> <div class="vector-toc-text"> <span class="vector-toc-numb">8</span> <span>Other animals</span> </div> </a> <ul id="toc-Other_animals-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Oral_manifestations" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Oral_manifestations"> <div class="vector-toc-text"> <span class="vector-toc-numb">9</span> <span>Oral manifestations</span> </div> </a> <ul id="toc-Oral_manifestations-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Dental_considerations" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Dental_considerations"> <div class="vector-toc-text"> <span class="vector-toc-numb">10</span> <span>Dental considerations</span> </div> </a> <ul id="toc-Dental_considerations-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-See_also" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#See_also"> <div class="vector-toc-text"> <span class="vector-toc-numb">11</span> <span>See also</span> </div> </a> <ul id="toc-See_also-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-References" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#References"> <div class="vector-toc-text"> <span class="vector-toc-numb">12</span> <span>References</span> </div> </a> <ul id="toc-References-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Further_reading" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Further_reading"> <div class="vector-toc-text"> <span class="vector-toc-numb">13</span> <span>Further reading</span> </div> </a> <ul id="toc-Further_reading-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-External_links" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#External_links"> <div class="vector-toc-text"> <span class="vector-toc-numb">14</span> <span>External links</span> </div> </a> <ul id="toc-External_links-sublist" class="vector-toc-list"> </ul> </li> </ul> </div> </div> </nav> </div> </div> <div class="mw-content-container"> <main id="content" class="mw-body"> <header class="mw-body-header vector-page-titlebar"> <nav aria-label="Contents" class="vector-toc-landmark"> <div id="vector-page-titlebar-toc" class="vector-dropdown vector-page-titlebar-toc vector-button-flush-left" > <input type="checkbox" id="vector-page-titlebar-toc-checkbox" role="button" aria-haspopup="true" data-event-name="ui.dropdown-vector-page-titlebar-toc" class="vector-dropdown-checkbox " aria-label="Toggle the table of contents" > <label id="vector-page-titlebar-toc-label" for="vector-page-titlebar-toc-checkbox" class="vector-dropdown-label cdx-button cdx-button--fake-button cdx-button--fake-button--enabled cdx-button--weight-quiet cdx-button--icon-only " aria-hidden="true" ><span class="vector-icon mw-ui-icon-listBullet mw-ui-icon-wikimedia-listBullet"></span> <span class="vector-dropdown-label-text">Toggle the table of contents</span> </label> <div class="vector-dropdown-content"> <div id="vector-page-titlebar-toc-unpinned-container" class="vector-unpinned-container"> </div> </div> </div> </nav> <h1 id="firstHeading" class="firstHeading mw-first-heading">von Willebrand disease</h1> <div id="p-lang-btn" class="vector-dropdown mw-portlet mw-portlet-lang" > <input type="checkbox" id="p-lang-btn-checkbox" role="button" aria-haspopup="true" data-event-name="ui.dropdown-p-lang-btn" class="vector-dropdown-checkbox mw-interlanguage-selector" aria-label="Go to an article in another language. Available in 30 languages" > <label id="p-lang-btn-label" for="p-lang-btn-checkbox" class="vector-dropdown-label cdx-button cdx-button--fake-button cdx-button--fake-button--enabled cdx-button--weight-quiet cdx-button--action-progressive mw-portlet-lang-heading-30" aria-hidden="true" ><span class="vector-icon mw-ui-icon-language-progressive mw-ui-icon-wikimedia-language-progressive"></span> <span class="vector-dropdown-label-text">30 languages</span> </label> <div class="vector-dropdown-content"> <div class="vector-menu-content"> <ul class="vector-menu-content-list"> <li class="interlanguage-link interwiki-ar mw-list-item"><a href="https://ar.wikipedia.org/wiki/%D9%85%D8%B1%D8%B6_%D9%81%D9%88%D9%86_%D9%88%D9%8A%D9%84%D9%8A%D8%A8%D8%B1%D8%A7%D9%86%D8%AF" title="مرض فون ويليبراند – Arabic" lang="ar" hreflang="ar" data-title="مرض فون ويليبراند" data-language-autonym="العربية" data-language-local-name="Arabic" class="interlanguage-link-target"><span>العربية</span></a></li><li class="interlanguage-link interwiki-az mw-list-item"><a href="https://az.wikipedia.org/wiki/Villebrand_x%C9%99st%C9%99liyi" title="Villebrand xəstəliyi – Azerbaijani" lang="az" hreflang="az" data-title="Villebrand xəstəliyi" data-language-autonym="Azərbaycanca" data-language-local-name="Azerbaijani" class="interlanguage-link-target"><span>Azərbaycanca</span></a></li><li class="interlanguage-link interwiki-bs mw-list-item"><a href="https://bs.wikipedia.org/wiki/Von_Willebrandova_bolest" title="Von Willebrandova bolest – Bosnian" lang="bs" hreflang="bs" data-title="Von Willebrandova bolest" data-language-autonym="Bosanski" data-language-local-name="Bosnian" class="interlanguage-link-target"><span>Bosanski</span></a></li><li class="interlanguage-link interwiki-ca mw-list-item"><a href="https://ca.wikipedia.org/wiki/Malaltia_de_von_Willebrand" title="Malaltia de von Willebrand – Catalan" lang="ca" hreflang="ca" data-title="Malaltia de von Willebrand" data-language-autonym="Català" data-language-local-name="Catalan" class="interlanguage-link-target"><span>Català</span></a></li><li class="interlanguage-link interwiki-de mw-list-item"><a href="https://de.wikipedia.org/wiki/Willebrand-J%C3%BCrgens-Syndrom" title="Willebrand-Jürgens-Syndrom – German" lang="de" hreflang="de" data-title="Willebrand-Jürgens-Syndrom" data-language-autonym="Deutsch" data-language-local-name="German" class="interlanguage-link-target"><span>Deutsch</span></a></li><li class="interlanguage-link interwiki-et mw-list-item"><a href="https://et.wikipedia.org/wiki/Von_Willebrandi_haigus" title="Von Willebrandi haigus – Estonian" lang="et" hreflang="et" data-title="Von Willebrandi haigus" data-language-autonym="Eesti" data-language-local-name="Estonian" class="interlanguage-link-target"><span>Eesti</span></a></li><li class="interlanguage-link interwiki-es mw-list-item"><a href="https://es.wikipedia.org/wiki/Enfermedad_de_Von_Willebrand" title="Enfermedad de Von Willebrand – Spanish" lang="es" hreflang="es" data-title="Enfermedad de Von Willebrand" data-language-autonym="Español" data-language-local-name="Spanish" class="interlanguage-link-target"><span>Español</span></a></li><li class="interlanguage-link interwiki-fa mw-list-item"><a href="https://fa.wikipedia.org/wiki/%D8%A8%DB%8C%D9%85%D8%A7%D8%B1%DB%8C_%D9%81%D9%88%D9%86%E2%80%8C%D9%88%DB%8C%D9%84%D8%A8%D8%B1%D8%A7%D9%86%D8%AF" title="بیماری فون‌ویلبراند – Persian" lang="fa" hreflang="fa" data-title="بیماری فون‌ویلبراند" data-language-autonym="فارسی" data-language-local-name="Persian" class="interlanguage-link-target"><span>فارسی</span></a></li><li class="interlanguage-link interwiki-fr mw-list-item"><a href="https://fr.wikipedia.org/wiki/Maladie_de_Willebrand" title="Maladie de Willebrand – French" lang="fr" hreflang="fr" data-title="Maladie de Willebrand" data-language-autonym="Français" data-language-local-name="French" class="interlanguage-link-target"><span>Français</span></a></li><li class="interlanguage-link interwiki-ko mw-list-item"><a href="https://ko.wikipedia.org/wiki/%ED%8F%B0_%EB%B9%8C%EB%A0%88%EB%B8%8C%EB%9E%80%ED%8A%B8_%EB%B3%91" title="폰 빌레브란트 병 – Korean" lang="ko" hreflang="ko" data-title="폰 빌레브란트 병" data-language-autonym="한국어" data-language-local-name="Korean" class="interlanguage-link-target"><span>한국어</span></a></li><li class="interlanguage-link interwiki-hr mw-list-item"><a href="https://hr.wikipedia.org/wiki/Von_Willebrandova_bolest" title="Von Willebrandova bolest – Croatian" lang="hr" hreflang="hr" data-title="Von Willebrandova bolest" data-language-autonym="Hrvatski" data-language-local-name="Croatian" class="interlanguage-link-target"><span>Hrvatski</span></a></li><li class="interlanguage-link interwiki-id mw-list-item"><a href="https://id.wikipedia.org/wiki/Penyakit_von_Willebrand" title="Penyakit von Willebrand – Indonesian" lang="id" hreflang="id" data-title="Penyakit von Willebrand" data-language-autonym="Bahasa Indonesia" data-language-local-name="Indonesian" class="interlanguage-link-target"><span>Bahasa Indonesia</span></a></li><li class="interlanguage-link interwiki-it mw-list-item"><a href="https://it.wikipedia.org/wiki/Malattia_di_von_Willebrand" title="Malattia di von Willebrand – Italian" lang="it" hreflang="it" data-title="Malattia di von Willebrand" data-language-autonym="Italiano" data-language-local-name="Italian" class="interlanguage-link-target"><span>Italiano</span></a></li><li class="interlanguage-link interwiki-he mw-list-item"><a href="https://he.wikipedia.org/wiki/%D7%9E%D7%97%D7%9C%D7%AA_%D7%A4%D7%95%D7%9F_%D7%95%D7%99%D7%9C%D7%91%D7%A8%D7%A0%D7%93" title="מחלת פון וילברנד – Hebrew" lang="he" hreflang="he" data-title="מחלת פון וילברנד" data-language-autonym="עברית" data-language-local-name="Hebrew" class="interlanguage-link-target"><span>עברית</span></a></li><li class="interlanguage-link interwiki-kk mw-list-item"><a href="https://kk.wikipedia.org/wiki/%D0%92%D0%B8%D0%BB%D0%BB%D0%B5%D0%B1%D1%80%D0%B0%D0%BD%D0%B4_%D0%B0%D1%83%D1%80%D1%83%D1%8B" title="Виллебранд ауруы – Kazakh" lang="kk" hreflang="kk" data-title="Виллебранд ауруы" data-language-autonym="Қазақша" data-language-local-name="Kazakh" class="interlanguage-link-target"><span>Қазақша</span></a></li><li class="interlanguage-link interwiki-hu mw-list-item"><a href="https://hu.wikipedia.org/wiki/Von_Willebrand%E2%80%93J%C3%BCrgens-szindr%C3%B3ma" title="Von Willebrand–Jürgens-szindróma – Hungarian" lang="hu" hreflang="hu" data-title="Von Willebrand–Jürgens-szindróma" data-language-autonym="Magyar" data-language-local-name="Hungarian" class="interlanguage-link-target"><span>Magyar</span></a></li><li class="interlanguage-link interwiki-mk mw-list-item"><a href="https://mk.wikipedia.org/wiki/%D0%A4%D0%BE%D0%BD_%D0%92%D0%B8%D0%BB%D0%B5%D0%B1%D1%80%D0%B0%D0%BD%D0%B4%D0%BE%D0%B2%D0%B0_%D0%B1%D0%BE%D0%BB%D0%B5%D1%81%D1%82" title="Фон Вилебрандова болест – Macedonian" lang="mk" hreflang="mk" data-title="Фон Вилебрандова болест" data-language-autonym="Македонски" data-language-local-name="Macedonian" class="interlanguage-link-target"><span>Македонски</span></a></li><li class="interlanguage-link interwiki-ms mw-list-item"><a href="https://ms.wikipedia.org/wiki/Penyakit_von_Willebrand" title="Penyakit von Willebrand – Malay" lang="ms" hreflang="ms" data-title="Penyakit von Willebrand" data-language-autonym="Bahasa Melayu" data-language-local-name="Malay" class="interlanguage-link-target"><span>Bahasa Melayu</span></a></li><li class="interlanguage-link interwiki-ja mw-list-item"><a href="https://ja.wikipedia.org/wiki/%E3%83%B4%E3%82%A9%E3%83%B3%E3%83%BB%E3%83%B4%E3%82%A3%E3%83%AC%E3%83%96%E3%83%A9%E3%83%B3%E3%83%89%E7%97%85" title="ヴォン・ヴィレブランド病 – Japanese" lang="ja" hreflang="ja" data-title="ヴォン・ヴィレブランド病" data-language-autonym="日本語" data-language-local-name="Japanese" class="interlanguage-link-target"><span>日本語</span></a></li><li class="interlanguage-link interwiki-no mw-list-item"><a href="https://no.wikipedia.org/wiki/Von_Willebrands_sykdom" title="Von Willebrands sykdom – Norwegian Bokmål" lang="nb" hreflang="nb" data-title="Von Willebrands sykdom" data-language-autonym="Norsk bokmål" data-language-local-name="Norwegian Bokmål" class="interlanguage-link-target"><span>Norsk bokmål</span></a></li><li class="interlanguage-link interwiki-pl mw-list-item"><a href="https://pl.wikipedia.org/wiki/Choroba_von_Willebranda" title="Choroba von Willebranda – Polish" lang="pl" hreflang="pl" data-title="Choroba von Willebranda" data-language-autonym="Polski" data-language-local-name="Polish" class="interlanguage-link-target"><span>Polski</span></a></li><li class="interlanguage-link interwiki-pt mw-list-item"><a href="https://pt.wikipedia.org/wiki/Doen%C3%A7a_de_von_Willebrand" title="Doença de von Willebrand – Portuguese" lang="pt" hreflang="pt" data-title="Doença de von Willebrand" data-language-autonym="Português" data-language-local-name="Portuguese" class="interlanguage-link-target"><span>Português</span></a></li><li class="interlanguage-link interwiki-ru mw-list-item"><a href="https://ru.wikipedia.org/wiki/%D0%91%D0%BE%D0%BB%D0%B5%D0%B7%D0%BD%D1%8C_%D0%92%D0%B8%D0%BB%D0%BB%D0%B5%D0%B1%D1%80%D0%B0%D0%BD%D0%B4%D0%B0" title="Болезнь Виллебранда – Russian" lang="ru" hreflang="ru" data-title="Болезнь Виллебранда" data-language-autonym="Русский" data-language-local-name="Russian" class="interlanguage-link-target"><span>Русский</span></a></li><li class="interlanguage-link interwiki-simple mw-list-item"><a href="https://simple.wikipedia.org/wiki/Von_Willebrand_disease" title="Von Willebrand disease – Simple English" lang="en-simple" hreflang="en-simple" data-title="Von Willebrand disease" data-language-autonym="Simple English" data-language-local-name="Simple English" class="interlanguage-link-target"><span>Simple English</span></a></li><li class="interlanguage-link interwiki-sr mw-list-item"><a href="https://sr.wikipedia.org/wiki/Fon_Vilebrandov_sindrom" title="Fon Vilebrandov sindrom – Serbian" lang="sr" hreflang="sr" data-title="Fon Vilebrandov sindrom" data-language-autonym="Српски / srpski" data-language-local-name="Serbian" class="interlanguage-link-target"><span>Српски / srpski</span></a></li><li class="interlanguage-link interwiki-fi mw-list-item"><a href="https://fi.wikipedia.org/wiki/Von_Willebrandin_tauti" title="Von Willebrandin tauti – Finnish" lang="fi" hreflang="fi" data-title="Von Willebrandin tauti" data-language-autonym="Suomi" data-language-local-name="Finnish" class="interlanguage-link-target"><span>Suomi</span></a></li><li class="interlanguage-link interwiki-sv mw-list-item"><a href="https://sv.wikipedia.org/wiki/Von_Willebrands_sjukdom" title="Von Willebrands sjukdom – Swedish" lang="sv" hreflang="sv" data-title="Von Willebrands sjukdom" data-language-autonym="Svenska" data-language-local-name="Swedish" 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<div class="mw-indicators"> </div> <div id="siteSub" class="noprint">From Wikipedia, the free encyclopedia</div> </div> <div id="contentSub"><div id="mw-content-subtitle"></div></div> <div id="mw-content-text" class="mw-body-content"><div class="mw-content-ltr mw-parser-output" lang="en" dir="ltr"><style data-mw-deduplicate="TemplateStyles:r1236090951">.mw-parser-output .hatnote{font-style:italic}.mw-parser-output div.hatnote{padding-left:1.6em;margin-bottom:0.5em}.mw-parser-output .hatnote i{font-style:normal}.mw-parser-output .hatnote+link+.hatnote{margin-top:-0.5em}@media print{body.ns-0 .mw-parser-output .hatnote{display:none!important}}</style><div role="note" class="hatnote navigation-not-searchable">"VWD" redirects here. For Visual Web Developer, see <a href="/wiki/Microsoft_Visual_Studio_Express#Visual_Web_Developer_Express" title="Microsoft Visual Studio Express">Microsoft Visual Studio Express § Visual Web Developer Express</a>.</div> <div class="shortdescription nomobile noexcerpt noprint searchaux" style="display:none">Medical condition</div><style data-mw-deduplicate="TemplateStyles:r1257001546">.mw-parser-output .infobox-subbox{padding:0;border:none;margin:-3px;width:auto;min-width:100%;font-size:100%;clear:none;float:none;background-color:transparent}.mw-parser-output .infobox-3cols-child{margin:auto}.mw-parser-output .infobox .navbar{font-size:100%}@media screen{html.skin-theme-clientpref-night .mw-parser-output .infobox-full-data:not(.notheme)>div:not(.notheme)[style]{background:#1f1f23!important;color:#f8f9fa}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .infobox-full-data:not(.notheme) div:not(.notheme){background:#1f1f23!important;color:#f8f9fa}}@media(min-width:640px){body.skin--responsive .mw-parser-output .infobox-table{display:table!important}body.skin--responsive .mw-parser-output .infobox-table>caption{display:table-caption!important}body.skin--responsive .mw-parser-output .infobox-table>tbody{display:table-row-group}body.skin--responsive .mw-parser-output .infobox-table tr{display:table-row!important}body.skin--responsive .mw-parser-output .infobox-table th,body.skin--responsive .mw-parser-output .infobox-table td{padding-left:inherit;padding-right:inherit}}</style><table class="infobox ib-medical-condition"><tbody><tr><th colspan="2" class="infobox-above" style="background:#ccc">von Willebrand disease</th></tr><tr style="background-color: #f8f9fa;"><td colspan="2" class="infobox-full-data"><span class="mw-default-size" typeof="mw:File/Frameless"><a href="/wiki/File:Autosomal_dominant_-_en.svg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/4/4e/Autosomal_dominant_-_en.svg/220px-Autosomal_dominant_-_en.svg.png" decoding="async" width="220" height="376" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/4/4e/Autosomal_dominant_-_en.svg/330px-Autosomal_dominant_-_en.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/4/4e/Autosomal_dominant_-_en.svg/440px-Autosomal_dominant_-_en.svg.png 2x" data-file-width="738" data-file-height="1260" /></a></span></td></tr><tr><td colspan="2" class="infobox-full-data">von Willebrand disease types I and II are inherited in an <a href="/wiki/Autosomal_dominant" class="mw-redirect" title="Autosomal dominant">autosomal dominant</a> pattern.</td></tr><tr><th scope="row" class="infobox-label">Pronunciation</th><td class="infobox-data"><style data-mw-deduplicate="TemplateStyles:r1126788409">.mw-parser-output .plainlist ol,.mw-parser-output .plainlist ul{line-height:inherit;list-style:none;margin:0;padding:0}.mw-parser-output .plainlist ol li,.mw-parser-output .plainlist ul li{margin-bottom:0}</style><div class="plainlist"><ul><li><span class="rt-commentedText nowrap"><span class="IPA nopopups noexcerpt" lang="en-fonipa"><a href="/wiki/Help:IPA/English" title="Help:IPA/English">/<span style="border-bottom:1px dotted"><span title="/ˌ/: secondary stress follows">ˌ</span><span title="'f' in 'find'">f</span><span title="/ʌ/: 'u' in 'cut'">ʌ</span><span title="'n' in 'nigh'">n</span><span title="/ˈ/: primary stress follows">ˈ</span><span title="'v' in 'vie'">v</span><span title="/ɪ/: 'i' in 'kit'">ɪ</span><span title="'l' in 'lie'">l</span><span title="/ɪ/: 'i' in 'kit'">ɪ</span><span title="'b' in 'buy'">b</span><span title="'r' in 'rye'">r</span><span title="/ɑː/: 'a' in 'father'">ɑː</span><span title="'n' in 'nigh'">n</span><span title="'t' in 'tie'">t</span></span>/</a></span></span> </li></ul></div></td></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Medical_specialty" title="Medical specialty">Specialty</a></th><td class="infobox-data"><a href="/wiki/Hematology" title="Hematology">Hematology</a></td></tr></tbody></table> <p><b>Von Willebrand disease</b> (<b>VWD</b>) is the most common <a href="/wiki/Heredity" title="Heredity">hereditary</a> <a href="/wiki/Coagulopathy" title="Coagulopathy">blood-clotting disorder</a> in humans. An acquired form can sometimes result from other medical conditions.<sup id="cite_ref-1" class="reference"><a href="#cite_note-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup> It arises from a deficiency in the quality or quantity of <a href="/wiki/Von_Willebrand_factor" title="Von Willebrand factor">von Willebrand factor</a> (VWF), a <a href="/wiki/Protein_subunit" title="Protein subunit">multimeric</a> protein that is required for <a href="/wiki/Platelet" title="Platelet">platelet</a> <a href="/wiki/Platelet#Adhesion" title="Platelet">adhesion</a>. It is known to affect several breeds of dogs as well as humans. The three forms of VWD are hereditary, acquired, and pseudo or platelet type. The three types of hereditary VWD are VWD type 1, VWD type 2, and VWD type 3. Type 2 contains various subtypes.<sup id="cite_ref-Kaur24_2-0" class="reference"><a href="#cite_note-Kaur24-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> Platelet type VWD is also an inherited condition.<sup id="cite_ref-SwystunJames2016_3-0" class="reference"><a href="#cite_note-SwystunJames2016-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> </p><p>In 2008 a new diagnostic category of "Low VWF" was proposed to include those individuals whose von Willebrand factor levels were in the 30–50 IU/dL range, below the normal reference range but not low enough to be von Willebrand disease.<sup id="cite_ref-4" class="reference"><a href="#cite_note-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> Patients with low VWF were sometimes noted to experience bleeding, despite mild reductions in VWF levels.<sup id="cite_ref-5" class="reference"><a href="#cite_note-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> The 2021 ASH/ISTH guidelines re-classified patients with levels in the 30–50 IU/dl range as "Low VWF" if they have no bleeding, but as having VWD if they have bleeding.<sup id="cite_ref-James21_6-0" class="reference"><a href="#cite_note-James21-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> </p><p>VWD type 1 is the most common type of the disorder, with mild bleeding symptoms such as <a href="/wiki/Nosebleed" title="Nosebleed">nosebleeds</a>, though occasionally more severe symptoms can occur. <a href="/wiki/Blood_type" title="Blood type">Blood type</a> can affect the presentation and severity of symptoms of VWD.<sup id="cite_ref-7" class="reference"><a href="#cite_note-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p><p>VWD type 2 is the second most common type of the disorder and has mild to moderate symptoms. </p><p>The factor is named after the Finnish physician <a href="/wiki/Erik_Adolf_von_Willebrand" title="Erik Adolf von Willebrand">Erik Adolf von Willebrand</a> who first described the condition in 1926. Guidelines for the diagnosis and management of VWD were updated in 2021.<sup id="cite_ref-James21_6-1" class="reference"><a href="#cite_note-James21-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Connell21_8-0" class="reference"><a href="#cite_note-Connell21-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> </p> <style data-mw-deduplicate="TemplateStyles:r886046785">.mw-parser-output .toclimit-2 .toclevel-1 ul,.mw-parser-output .toclimit-3 .toclevel-2 ul,.mw-parser-output .toclimit-4 .toclevel-3 ul,.mw-parser-output .toclimit-5 .toclevel-4 ul,.mw-parser-output .toclimit-6 .toclevel-5 ul,.mw-parser-output .toclimit-7 .toclevel-6 ul{display:none}</style><div class="toclimit-3"><meta property="mw:PageProp/toc" /></div> <div class="mw-heading mw-heading2"><h2 id="Signs_and_symptoms">Signs and symptoms</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=1" title="Edit section: Signs and symptoms"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The various types of VWD present with varying degrees of <a href="/wiki/Bleeding_tendency" class="mw-redirect" title="Bleeding tendency">bleeding tendency</a>, usually in the form of easy <a href="/wiki/Bruise" title="Bruise">bruising</a>, <a href="/wiki/Nosebleed" title="Nosebleed">nosebleeds</a>, and <a href="/wiki/Bleeding_on_probing" title="Bleeding on probing">bleeding gums</a>.<sup id="cite_ref-Kaur24_2-1" class="reference"><a href="#cite_note-Kaur24-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> Women may experience <a href="/wiki/Menorrhagia" class="mw-redirect" title="Menorrhagia">heavy menstrual periods</a> and <a href="/wiki/Blood_loss" class="mw-redirect" title="Blood loss">blood loss</a> during <a href="/wiki/Childbirth#Maternal_complications" title="Childbirth">childbirth.</a><sup id="cite_ref-9" class="reference"><a href="#cite_note-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-10" class="reference"><a href="#cite_note-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> </p><p>Severe <a href="/wiki/Internal_bleeding" title="Internal bleeding">internal bleeding</a> and <a href="/wiki/Hemarthrosis" title="Hemarthrosis">bleeding into joints</a> are uncommon in all but the most severe type, VWD type 3. </p> <div class="mw-heading mw-heading2"><h2 id="Genetics">Genetics</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=2" title="Edit section: Genetics"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The VWF gene is located on the <a href="/wiki/Locus_(genetics)" title="Locus (genetics)">short arm <i>p</i></a> of <a href="/wiki/Chromosome_12_(human)" class="mw-redirect" title="Chromosome 12 (human)">chromosome 12</a> (12p13.2). It has 52 <a href="/wiki/Exons" class="mw-redirect" title="Exons">exons</a> spanning 178 <a href="/wiki/Kilobase" class="mw-redirect" title="Kilobase">kbp</a>. Types 1 and 2 are inherited as <a href="/wiki/Autosomal_dominant" class="mw-redirect" title="Autosomal dominant">autosomal dominant</a> traits. Occasionally, type 2 also inherits recessively. Type 3 is inherited as <a href="/wiki/Autosomal_recessive" class="mw-redirect" title="Autosomal recessive">autosomal recessive</a>. However, some individuals heterozygous for type 3 may be diagnosed with VWD type 1, indicating an intermediate inheritance in those cases. VWD occurs in approximately 1% of the population and affects men and women equally.<sup id="cite_ref-auto_11-0" class="reference"><a href="#cite_note-auto-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup> </p><p><a href="/wiki/Genetic_testing" title="Genetic testing">Genetic testing</a> is typically not part of the initial workup for von Willebrand disease, and is not needed for people diagnosed with type 1 VWD based on clinical history and laboratory tests.<sup id="cite_ref-arup_12-0" class="reference"><a href="#cite_note-arup-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> It is mainly useful for:<sup id="cite_ref-arup_12-1" class="reference"><a href="#cite_note-arup-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p> <ul><li>Evaluating family members of individuals who have known variants.</li> <li>Differentiating between type 2B and platelet-type VWD, as well as between type 2N VWD and <a href="/wiki/Hemophilia_A" class="mw-redirect" title="Hemophilia A">hemophilia A</a>.</li></ul> <div class="mw-heading mw-heading2"><h2 id="Pathophysiology">Pathophysiology</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=3" title="Edit section: Pathophysiology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236090951"><div role="note" class="hatnote navigation-not-searchable">For the normal function of the coagulation factor, see <a href="/wiki/Von_Willebrand_factor" title="Von Willebrand factor">von Willebrand factor</a>.</div> <p><a href="/wiki/Von_Willebrand_factor" title="Von Willebrand factor">Von Willebrand factor</a> is mainly active in conditions of high blood flow and <a href="/wiki/Shear_stress" title="Shear stress">shear stress</a>. Deficiency of VWF, therefore, shows primarily in organs with extensive <a href="/wiki/Capillary" title="Capillary">small vessels</a>, such as <a href="/wiki/Human_skin" title="Human skin">skin</a>, <a href="/wiki/Human_gastrointestinal_tract" class="mw-redirect" title="Human gastrointestinal tract">gastrointestinal tract</a>, and <a href="/wiki/Uterus" title="Uterus">uterus</a>. In <a href="/wiki/Angiodysplasia" title="Angiodysplasia">angiodysplasia</a>, a form of <a href="/wiki/Telangiectasia" title="Telangiectasia">telangiectasia</a> of the <a href="/wiki/Large_intestine#Structure" title="Large intestine">colon</a>, shear stress is much higher than in average <a href="/wiki/Capillary" title="Capillary">capillaries</a>, and the risk of bleeding is increased concomitantly.<sup id="cite_ref-13" class="reference"><a href="#cite_note-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> </p><p>In more severe cases of type 1 VWD, genetic changes are common within the VWF gene and are highly <a href="/wiki/Penetrance" title="Penetrance">penetrant</a>. In milder cases of type 1 VWD, a complex spectrum of molecular <a href="/wiki/Pathology" title="Pathology">pathology</a> may exist in addition to <a href="/wiki/Polymorphism_(biology)" title="Polymorphism (biology)">polymorphisms</a> of the VWF gene alone.<sup id="cite_ref-14" class="reference"><a href="#cite_note-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> </p><p>The individual's <a href="/wiki/ABO_blood_group_system#Bleeding_and_thrombosis_(von_Willebrand_factor)" title="ABO blood group system">ABO blood group</a> can influence presentation and pathology of VWD. Those individuals with <a href="/wiki/Blood_type" title="Blood type">blood group O</a> have a lower mean level than individuals with other blood groups. Unless ABO group-specific VWF:antigen reference ranges are used, normal group O individuals can be diagnosed as type I VWD, and some individuals of blood group AB with a genetic defect of VWF may have the diagnosis overlooked because VWF levels are elevated due to blood group.<sup id="cite_ref-15" class="reference"><a href="#cite_note-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Diagnosis">Diagnosis</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=4" title="Edit section: Diagnosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Basic tests performed in any patient with bleeding problems are a <a href="/wiki/Complete_blood_count" title="Complete blood count">complete blood count</a>-CBC (especially <a href="/wiki/Platelet" title="Platelet">platelet</a> counts), <a href="/wiki/APTT" class="mw-redirect" title="APTT">activated partial thromboplastin time</a>-APTT, <a href="/wiki/Prothrombin_time" title="Prothrombin time">prothrombin time</a> with International Normalized Ratio-PTINR, <a href="/wiki/Thrombin" title="Thrombin">thrombin</a> time-TT, and <a href="/wiki/Fibrinogen" title="Fibrinogen">fibrinogen</a> level. Patients with abnormal tests typically undergo further testing for hemophilias. Other <a href="/wiki/Coagulation_factor" class="mw-redirect" title="Coagulation factor">coagulation factor</a> assays may be performed depending on the results of a coagulation screen. Patients with von Willebrand disease typically display a normal prothrombin time and a variable prolongation of APTT, depending on whether sufficient VWF is available to perform its carrier function for factor VIII.<sup id="cite_ref-James21_6-2" class="reference"><a href="#cite_note-James21-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> </p><p>When VWD is suspected, <a href="/wiki/Blood_plasma" title="Blood plasma">blood plasma</a> of a patient must be investigated for quantitative and qualitative deficiencies of VWF. This is achieved by measuring the amount of VWF in a VWF antigen assay and the functionality of VWF with a <a href="/wiki/Glycoprotein" title="Glycoprotein">glycoprotein</a> (GP)Ib binding assay, VWF antibody assay, or a <a href="/wiki/Ristocetin" title="Ristocetin">ristocetin</a> cofactor activity (RiCof) assay.<sup id="cite_ref-16" class="reference"><a href="#cite_note-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup> <a href="/wiki/Factor_VIII" title="Factor VIII">Factor VIII</a> levels are also performed because factor VIII is bound to VWF which protects the factor VIII from rapid breakdown within the blood. Deficiency of VWF can then lead to a reduction in factor VIII levels, which explains the elevation in PTT. Normal levels do not exclude all forms of VWD, particularly type 2, which may only be revealed by investigating platelet interaction with subendothelium under flow, a highly specialized coagulation study not routinely performed in most <a href="/wiki/Medical_laboratory" title="Medical laboratory">medical laboratories</a>. <a href="/wiki/Ristocetin-induced_platelet_agglutination" class="mw-redirect" title="Ristocetin-induced platelet agglutination">Ristocetin-induced platelet agglutination</a> (RIPA), collagen binding, and/or VWF multimer assays may be performed to follow up abnormal screening tests. A platelet aggregation assay will show an abnormal response to ristocetin with normal responses to the other agonists used: </p> <table class="wikitable" align="center"> <caption><a href="/wiki/Platelet#Tests_of_function" title="Platelet">Platelet aggregation function</a> by main disorders and agonists   <span class="noprint plainlinks" title="Edit Template:Table of platelet aggregation disorders and agonists"><a class="external text" href="https://en.wikipedia.org/w/index.php?title=Template:Table_of_platelet_aggregation_disorders_and_agonists&action=edit">edit</a></span> <link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236090951"><div role="note" class="hatnote navigation-not-searchable">Further information: <a href="/wiki/Platelet#Tests_of_function" title="Platelet">Platelet § Tests of function</a></div> </caption> <tbody><tr> <th></th> <th><a href="/wiki/Adenosine_diphosphate" title="Adenosine diphosphate">ADP</a></th> <th><a href="/wiki/Epinephrine_(medication)" title="Epinephrine (medication)">Epinephrine</a></th> <th><a href="/wiki/Collagen" title="Collagen">Collagen</a></th> <th><a href="/wiki/Ristocetin" title="Ristocetin">Ristocetin</a> </th></tr> <tr> <th><a href="/wiki/P2Y_receptor" title="P2Y receptor">P2Y receptor</a> inhibitor or defect<sup id="cite_ref-BorhanyPahore2010_17-0" class="reference"><a href="#cite_note-BorhanyPahore2010-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> </th> <td><b>Decreased</b></td> <td>Normal</td> <td>Normal</td> <td>Normal </td></tr> <tr> <th><a href="/wiki/Adrenergic_receptor" title="Adrenergic receptor">Adrenergic receptor</a> defect<sup id="cite_ref-BorhanyPahore2010_17-1" class="reference"><a href="#cite_note-BorhanyPahore2010-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> </th> <td>Normal</td> <td><b>Decreased</b></td> <td>Normal</td> <td>Normal </td></tr> <tr> <th><a href="/wiki/Collagen_receptor" title="Collagen receptor">Collagen receptor</a> defect<sup id="cite_ref-BorhanyPahore2010_17-2" class="reference"><a href="#cite_note-BorhanyPahore2010-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> </th> <td>Normal</td> <td>Normal</td> <td><b>Decreased or absent</b></td> <td>Normal </td></tr> <tr> <th> <ul><li><a class="mw-selflink selflink">Von Willebrand disease</a> (except Type 2B)</li> <li><a href="/wiki/Bernard%E2%80%93Soulier_syndrome" title="Bernard–Soulier syndrome">Bernard–Soulier syndrome</a><sup id="cite_ref-BorhanyPahore2010_17-3" class="reference"><a href="#cite_note-BorhanyPahore2010-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup></li></ul> </th> <td>Normal</td> <td>Normal</td> <td>Normal</td> <td><b>Decreased or absent</b> </td></tr> <tr> <th> <ul><li><a href="/wiki/Glanzmann%27s_thrombasthenia" title="Glanzmann's thrombasthenia">Glanzmann's thrombasthenia</a><sup id="cite_ref-BorhanyPahore2010_17-4" class="reference"><a href="#cite_note-BorhanyPahore2010-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup></li></ul> </th> <td><b>Decreased</b></td> <td><b>Decreased</b></td> <td><b>Decreased</b></td> <td>Normal or <b>decreased</b> </td></tr> </tbody></table> <p>A <a href="/wiki/PFA-100" title="PFA-100">platelet function assay</a> may give an abnormal collagen/<a href="/wiki/Epinephrine" class="mw-redirect" title="Epinephrine">epinephrine</a> closure time, and in most cases, a normal collagen/<a href="/wiki/Adenosine_diphosphate" title="Adenosine diphosphate">ADP</a> time. Type 2N may be considered if factor VIII levels are disproportionately low, but confirmation requires a "factor VIII binding" assay. Additional laboratory tests that help classify sub-types of VWD include von Willebrand multimer analysis, modified ristocetin induced platelet aggregation assay and VWF propeptide to VWF propeptide antigen ratio. In cases of suspected acquired von Willebrand syndrome, a mixing study (analysis of patient plasma along with pooled normal plasma/PNP and a mixture of the two tested immediately, at one hour, and at two hours) should be performed.<sup id="cite_ref-18" class="reference"><a href="#cite_note-18"><span class="cite-bracket">[</span>18<span class="cite-bracket">]</span></a></sup> Detection of VWD is complicated by VWF being an <a href="/wiki/Acute-phase_reactant" class="mw-redirect" title="Acute-phase reactant">acute-phase reactant</a> with levels rising in infection, pregnancy, and stress. </p><p>The testing for VWD can be influenced by laboratory procedures. Numerous variables exist in the testing procedure that may affect the validity of the test results and may result in a missed or erroneous diagnosis. The chance of procedural errors are typically greatest during the preanalytical phase (during collecting storage and transportation of the specimen) especially when the testing is contracted to an outside facility and the specimen is frozen and transported long distances.<sup id="cite_ref-19" class="reference"><a href="#cite_note-19"><span class="cite-bracket">[</span>19<span class="cite-bracket">]</span></a></sup> Diagnostic errors are not uncommon, and the rate of testing proficiency varies amongst laboratories, with error rates ranging from 7 to 22% in some studies to as high as 60% in cases of misclassification of VWD subtype. To increase the probability of a proper diagnosis, testing should be done at a facility with immediate on-site processing in a specialized coagulation laboratory.<sup id="cite_ref-20" class="reference"><a href="#cite_note-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-21" class="reference"><a href="#cite_note-21"><span class="cite-bracket">[</span>21<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Types">Types</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=5" title="Edit section: Types"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <figure class="mw-default-size" typeof="mw:File/Thumb"><a href="/wiki/File:Pie_chart_of_relative_incidences_of_Von_Willebrand_disease_types.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/f/f5/Pie_chart_of_relative_incidences_of_Von_Willebrand_disease_types.jpg/220px-Pie_chart_of_relative_incidences_of_Von_Willebrand_disease_types.jpg" decoding="async" width="220" height="184" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/f/f5/Pie_chart_of_relative_incidences_of_Von_Willebrand_disease_types.jpg/330px-Pie_chart_of_relative_incidences_of_Von_Willebrand_disease_types.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/f/f5/Pie_chart_of_relative_incidences_of_Von_Willebrand_disease_types.jpg/440px-Pie_chart_of_relative_incidences_of_Von_Willebrand_disease_types.jpg 2x" data-file-width="685" data-file-height="573" /></a><figcaption>Pie chart of relative incidences of von Willebrand disease types in <a href="/wiki/South_Africa" title="South Africa">South Africa</a>. Platelet-type was <0.5% of cases.<sup id="cite_ref-Meiring2011_22-0" class="reference"><a href="#cite_note-Meiring2011-22"><span class="cite-bracket">[</span>22<span class="cite-bracket">]</span></a></sup></figcaption></figure> <p>The four hereditary types of VWD described are type 1, type 2, type 3, and pseudo- or platelet-type. Most cases are hereditary, but acquired forms of VWD have been described. The <a href="/wiki/International_Society_on_Thrombosis_and_Haemostasis" title="International Society on Thrombosis and Haemostasis">International Society on Thrombosis and Haemostasis</a>'s classification depends on the definition of qualitative and quantitative defects.<sup id="cite_ref-James21_6-3" class="reference"><a href="#cite_note-James21-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> </p> <figure class="mw-default-size mw-halign-right" typeof="mw:File/Thumb"><a href="/wiki/File:Autorecessive.svg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/3/3e/Autorecessive.svg/220px-Autorecessive.svg.png" decoding="async" width="220" height="257" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/3/3e/Autorecessive.svg/330px-Autorecessive.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/3/3e/Autorecessive.svg/440px-Autorecessive.svg.png 2x" data-file-width="1350" data-file-height="1580" /></a><figcaption>von Willebrand disease type III (and sometimes II) is inherited in an <a href="/wiki/Autosomal_recessive" class="mw-redirect" title="Autosomal recessive">autosomal recessive</a> pattern.</figcaption></figure> <div class="mw-heading mw-heading4"><h4 id="Type_1">Type 1</h4><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=6" title="Edit section: Type 1"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Type 1 VWD (40<sup id="cite_ref-Meiring2011_22-1" class="reference"><a href="#cite_note-Meiring2011-22"><span class="cite-bracket">[</span>22<span class="cite-bracket">]</span></a></sup>-80%<sup id="cite_ref-auto_11-1" class="reference"><a href="#cite_note-auto-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup> of all VWD cases) is a quantitative defect which is <a href="/wiki/Heterozygous" class="mw-redirect" title="Heterozygous">heterozygous</a> for the defective gene. It arises from failure to secrete VWF into the circulation or, in the case of Type 1C, from VWF being cleared more quickly than normal. If VWF levels are greater than 50%, VWD can be ruled out. If VWF activity is below 30%, VWD is present. Patients with VWF activity between 0.30-0.50 IU/mL are classified based on their bleeding phenotype. If they have bleeding symptoms, they have VWD. If they have no bleeding, they are considered to have "Low VWF".<sup id="cite_ref-James21_6-4" class="reference"><a href="#cite_note-James21-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> </p><p>Many patients are asymptomatic or may have mild symptoms and not have clearly impaired <a href="/wiki/Coagulation" title="Coagulation">clotting</a>, which might suggest a bleeding disorder. Often, the discovery of low VWF occurs <a href="/wiki/Incidental_findings" class="mw-redirect" title="Incidental findings">incidentally</a> to other medical procedures requiring a blood work-up. Most cases of low VWF are never diagnosed due to its asymptomatic or mild presentation, and most people with type I VWD end up leading a normal life free of complications, with many being unaware that they have the disorder. </p><p>Trouble may, however, arise in some patients in the form of bleeding following surgery (including dental procedures), noticeable easy bruising, or <a href="/wiki/Menorrhagia" class="mw-redirect" title="Menorrhagia">menorrhagia</a> (heavy menstrual periods). The minority of cases of type 1 may present with severe hemorrhagic symptoms. </p> <div class="mw-heading mw-heading4"><h4 id="Type_1C">Type 1C</h4><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=7" title="Edit section: Type 1C"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Type 1C VWD indicates patients with quantitative deficiency due to an enhanced VWF clearance, accounting for ∼15% to 20% of cases. Such patients may require VWF concentrate to treat/prevent bleeds.<sup id="cite_ref-23" class="reference"><a href="#cite_note-23"><span class="cite-bracket">[</span>23<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-James21_6-5" class="reference"><a href="#cite_note-James21-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading4"><h4 id="Type_2">Type 2</h4><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=8" title="Edit section: Type 2"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Type 2 VWD (15<sup id="cite_ref-auto_11-2" class="reference"><a href="#cite_note-auto-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup>-50%<sup id="cite_ref-Meiring2011_22-2" class="reference"><a href="#cite_note-Meiring2011-22"><span class="cite-bracket">[</span>22<span class="cite-bracket">]</span></a></sup> of cases) is a qualitative defect and the bleeding tendency can vary between individuals. Four subtypes exist: 2A, 2B, 2M, and 2N. These subtypes depend on the presence and behavior of the underlying multimers.<sup id="cite_ref-auto_11-3" class="reference"><a href="#cite_note-auto-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup> Type 2 VWD (other than 2N) features an activity-to-antigen ratio of less than 0.7.<sup id="cite_ref-James21_6-6" class="reference"><a href="#cite_note-James21-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> This ratio is obtained by dividing the VWF activity by the VWF antigen. </p> <div class="mw-heading mw-heading5"><h5 id="Type_2A">Type 2A</h5><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=9" title="Edit section: Type 2A"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>VWD Type 2A results from a <a href="/wiki/Mutation" title="Mutation">loss-of-function mutation</a> in <a href="/wiki/Von_Willebrand_factor" title="Von Willebrand factor">von Willebrand factor (VWF)</a>, leading to reduced binding of VWF with Platelet <a href="/wiki/GP1BA" title="GP1BA">GP1b</a> receptors. This disorder follows an <a href="/wiki/Autosomal_dominant" class="mw-redirect" title="Autosomal dominant">autosomal dominant</a> inheritance pattern with only occasional cases following autosomal recessive pattern. It accounts for 10-15% of all VWD cases. It manifests with moderate to moderately severe bleeding. Diagnostic features of VWD Type 2A include greater reduction in VWF activity (measured by a functional assay) compared to VWF antigen (quantity). This results in a decreased VWF Activity to Antigen ratio. Additionally, high molecular weight multimers are either absent or very low on <a href="/wiki/Electrophoresis" title="Electrophoresis">electrophoresis</a> due to impaired multimer assembly or increased susceptibility to <a href="/wiki/ADAMTS13" title="ADAMTS13">ADAMTS13</a> (a protease that cleaves VWF). Factor VIII activity can be normal or low. <a href="/wiki/Ristocetin-induced_platelet_aggregation" title="Ristocetin-induced platelet aggregation">Ristocetin-induced platelet aggregation (RIPA)</a> is typically low. </p> <div class="mw-heading mw-heading5"><h5 id="Type_2B">Type 2B</h5><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=10" title="Edit section: Type 2B"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>This is a "<a href="/wiki/Mutation" title="Mutation">gain of function</a>" defect. The ability of the qualitatively defective VWF to bind to <a href="/wiki/Glycoprotein_Ib" title="Glycoprotein Ib">glycoprotein Ib</a> (GPIb) receptor on the platelet membrane is abnormally enhanced, leading to its spontaneous binding to platelets and subsequent rapid clearance of the bound platelets and of the large VWF multimers. Thrombocytopenia may occur. Large VWF multimers are reduced or absent from the circulation.<sup id="cite_ref-James21_6-7" class="reference"><a href="#cite_note-James21-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> </p><p>The ristocetin cofactor activity is low when the patient's platelet-poor plasma is assayed against formalin-fixed, normal donor platelets. However, when the assay is performed with the patient's own platelets (platelet-rich plasma), a lower-than-normal amount of ristocetin causes aggregation to occur. This is due to the large VWF multimers remaining bound to the patient's platelets. Patients with this subtype are unable to use desmopressin as a treatment for bleeding, because it can lead to unwanted platelet aggregation and aggravation of thrombocytopenia.<sup id="cite_ref-Desmopressin_24-0" class="reference"><a href="#cite_note-Desmopressin-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading5"><h5 id="Type_2M">Type 2M</h5><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=11" title="Edit section: Type 2M"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>VWD Type 2M results from a <a href="/wiki/Loss-of-function_mutation" class="mw-redirect" title="Loss-of-function mutation">loss-of-function mutation</a> in <a href="/wiki/Von_Willebrand_factor" title="Von Willebrand factor">von Willebrand factor (VWF)</a>. This mutation leads to reduced binding of VWF with <a href="/wiki/GP1BA" title="GP1BA">GP1b</a> (similar to VWD Type 2A) or with collagen. Like other Type 2 VWD subtypes, there is a decreased ratio of VWF Activity to antigen. Differentiating VWD Type 2M from Type 2A involves analyzing VWF multimers through <a href="/wiki/Gel_electrophoresis" title="Gel electrophoresis">electrophoresis</a>. In VWD Type 2M, all multimers are identified but uniformly decreased in quantity, resembling the pattern seen in VWD Type 1. Conversely, in VWD Type 2A, high molecular weight multimers are either absent or present in very low quantities. VWD Type 2M can be further differentiated from VWD Type 1 based on the VWF Activity to antigen ratio. In Type 1, the ratio is >0.7.<sup id="cite_ref-James21_6-8" class="reference"><a href="#cite_note-James21-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> In Type 2M, the ratio is <0.7. In Type 2M, factor VIII activity can be normal or low while the <a href="/wiki/Ristocetin-induced_platelet_aggregation" title="Ristocetin-induced platelet aggregation">ristocetin-induced platelet aggregation (RIPA)</a> is typically low. It is uncommon and manifests with moderate to severe bleeding. The disease may follow either an <a href="/wiki/Autosomal_dominant" class="mw-redirect" title="Autosomal dominant">autosomal dominant</a> or <a href="/wiki/Recessive" class="mw-redirect" title="Recessive">recessive</a> pattern of inheritance. </p> <div class="mw-heading mw-heading5"><h5 id="Type_2N_(Normandy)"><span id="Type_2N_.28Normandy.29"></span>Type 2N (Normandy)</h5><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=12" title="Edit section: Type 2N (Normandy)"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Type 2N VWD results from a <a href="/wiki/Loss_of_function" class="mw-redirect" title="Loss of function">loss-of-function mutation</a> that reduces the binding of <a href="/wiki/Von_Willebrand_factor" title="Von Willebrand factor">von Willebrand factor</a> (VWF) to factor VIII. Although VWF antigen (quantity) and activity levels (<a href="/wiki/Ristocetin" title="Ristocetin">Ristocetin</a> cofactor assay) remain normal, <a href="/wiki/Factor_VIII" title="Factor VIII">factor VIII</a> levels are typically low (usually 5-15%) due to impaired VWF binding. This vulnerability to proteolysis in the circulation leads to clinical manifestations resembling those of <a href="/wiki/Haemophilia_A" title="Haemophilia A">Hemophilia A</a>. The significantly reduced factor VIII levels in VWD Type 2N can sometimes lead to misdiagnosis as mild Hemophilia A. Like Hemophilia A, VWD Type 2N presents with joint and soft tissue bleeds. It is an autosomal recessive disorder, requiring either homozygosity or double heterozygosity for disease manifestation. Diagnostic tools include assessing the ratio of VWF binding to VWF antigen levels.<sup id="cite_ref-25" class="reference"><a href="#cite_note-25"><span class="cite-bracket">[</span>25<span class="cite-bracket">]</span></a></sup> A ratio <0.3 indicates <a href="/wiki/Homozygous" class="mw-redirect" title="Homozygous">homozygous</a> or double heterozygous VWD Type 2N, while a ratio <0.5 suggests <a href="/wiki/Heterozygous" class="mw-redirect" title="Heterozygous">heterozygous</a> VWD Type 2N. Conversely, a VWF antigen-to-binding ratio >3 confirms the diagnosis of VWD Type 2N. Ristocetin-Induced Platelet Agglutination (<a href="/wiki/Ristocetin-induced_platelet_aggregation" title="Ristocetin-induced platelet aggregation">RIPA</a>) and VWF multimer analysis are typically normal. </p> <div class="mw-heading mw-heading4"><h4 id="Type_3">Type 3</h4><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=13" title="Edit section: Type 3"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>VWD type 3 is a rare but the most severe form of VWD. It occurs in individuals who are <a href="/wiki/Homozygous" class="mw-redirect" title="Homozygous">homozygous</a> for the defective <a href="/wiki/Gene" title="Gene">gene</a>, resulting in a severe quantitative deficiency or complete absence of <a href="/wiki/Von_Willebrand_factor" title="Von Willebrand factor">von Willebrand factor (VWF)</a> production. In VWD type 3, VWF is undetectable in the VWF antigen assay. Since VWF normally protects coagulation <a href="/wiki/Factor_VIII" title="Factor VIII">factor VIII</a> from <a href="/wiki/Proteolysis" title="Proteolysis">proteolytic</a> degradation, the total absence of VWF leads to extremely low factor VIII levels (typically 1-10%). These low levels are equivalent to those seen in <a href="/wiki/Haemophilia_A" title="Haemophilia A">severe hemophilia A</a>, with clinical manifestations of life-threatening external and internal <a href="/wiki/Bleeding" title="Bleeding">hemorrhages</a>. The inheritance pattern of VWD type 3 is <a href="/wiki/Autosomal_recessive" class="mw-redirect" title="Autosomal recessive">autosomal recessive</a>, meaning that both parents must carry the defective gene for their child to be affected. In contrast, hemophilia A follows an <a href="/wiki/X-linked_recessive_inheritance" title="X-linked recessive inheritance">X-linked recessive</a> inheritance pattern. Additional diagnostic tools for VWD type 3 include assessing VWF activity using the Ristocetin cofactor assay and Collagen binding assay. In VWD type 3, VWF activity is either absent or approaching undetectable. VWF multimer analysis reveals no bands or very faint bands on electrophoresis. Additionally, Ristocetin-Induced Platelet Agglutination (<a href="/wiki/Ristocetin-induced_platelet_aggregation" title="Ristocetin-induced platelet aggregation">RIPA</a>) is typically absent or severely low. </p> <div class="mw-heading mw-heading4"><h4 id="Comparison">Comparison</h4><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=14" title="Edit section: Comparison"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <table class="wikitable"> <caption>Von Willebrand disease types<sup id="cite_ref-Rick_26-0" class="reference"><a href="#cite_note-Rick-26"><span class="cite-bracket">[</span>26<span class="cite-bracket">]</span></a></sup> </caption> <tbody><tr> <th colspan="2"> </th> <th>Mechanism</th> <th><a href="/wiki/Autosome" title="Autosome">Autosomal</a> inheritance</th> <th>vWF activity</th> <th><a href="/wiki/Ristocetin-induced_platelet_aggregation" title="Ristocetin-induced platelet aggregation">RIPA</a></th> <th>Multimer quantity </th></tr> <tr> <th colspan="2">Type 1 </th> <td>Decreased VWF quantity </td> <td>Dominant</td> <td>Decreased</td> <td>Normal<sup id="cite_ref-arup_12-2" class="reference"><a href="#cite_note-arup-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> or decreased<sup id="cite_ref-Rick_26-1" class="reference"><a href="#cite_note-Rick-26"><span class="cite-bracket">[</span>26<span class="cite-bracket">]</span></a></sup></td> <td>Similar decrease among multimer types </td></tr> <tr> <th rowspan="4">Type 2 </th> <td>A </td> <td>Inability to form large multimers</td> <td>Dominant or recessive</td> <td>Decreased</td> <td>Often decreased at high ristocetin concentrations<sup id="cite_ref-arup_12-3" class="reference"><a href="#cite_note-arup-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup></td> <td>Decreased large multimers </td></tr> <tr> <td>B </td> <td>Enhanced binding to GPIb receptor</td> <td>Dominant</td> <td>Decreased</td> <td><b>Increased</b></td> <td>Decreased large multimers </td></tr> <tr> <td>M </td> <td>Decreased binding to GPIb receptor</td> <td>Dominant or recessive</td> <td>Decreased</td> <td>Decreased at high ristocetin concentrations<sup id="cite_ref-arup_12-4" class="reference"><a href="#cite_note-arup-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup></td> <td>Similar decrease among multimer types </td></tr> <tr> <td>N </td> <td>Decreased binding to <a href="/wiki/Factor_VIII" title="Factor VIII">factor VIII</a></td> <td>Recessive</td> <td>Normal</td> <td>Normal</td> <td>Normal </td></tr> <tr> <th colspan="2">Type 3 </th> <td>Absent VWF </td> <td>Recessive</td> <td>Absent or markedly decreased</td> <td>Absent or markedly decreased</td> <td>Usually undetectable </td></tr></tbody></table> <div class="mw-heading mw-heading4"><h4 id="Platelet-type">Platelet-type</h4><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=15" title="Edit section: Platelet-type"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Platelet-type VWD (also known as pseudo-VWD) is an autosomal dominant genetic defect of the platelets. The VWF is qualitatively normal and genetic testing of the von Willebrand gene and VWF protein reveals no mutational alteration. The defect lies in the qualitatively altered GPIb receptor on the platelet membrane which increases its affinity to bind to the VWF. Large platelet aggregates and high molecular weight VWF multimers are removed from the circulation resulting in thrombocytopenia and diminished or absent large VWF multimers. The ristocetin cofactor activity and loss of large VWF multimers are similar to VWD type 2B. </p> <div class="mw-heading mw-heading4"><h4 id="Acquired">Acquired</h4><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=16" title="Edit section: Acquired"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Acquired Von Willebrand syndrome can occur due to <a href="/wiki/Autoantibody" title="Autoantibody">autoantibodies</a>, either interfering with platelet or collagen binding; increasing VWF clearance from the plasma; adsorption to myeloma cells or platelets; or proteolytic cleavage of VWF after shear stress-induced unfolding.<sup id="cite_ref-27" class="reference"><a href="#cite_note-27"><span class="cite-bracket">[</span>27<span class="cite-bracket">]</span></a></sup> </p><p>A form of acquired VWD occurs in patients with <a href="/wiki/Aortic_valve_stenosis" class="mw-redirect" title="Aortic valve stenosis">aortic valve stenosis</a>, leading to <a href="/wiki/Gastrointestinal_bleeding" title="Gastrointestinal bleeding">gastrointestinal bleeding</a> (<a href="/wiki/Heyde%27s_syndrome" title="Heyde's syndrome">Heyde's syndrome</a>). This form of acquired VWD may be more prevalent than is presently thought. In 2003, Vincentelli <i>et al.</i> noted that patients with acquired VWD and aortic stenosis who underwent valve replacement experienced a correction of their hemostatic abnormalities, but that the hemostatic abnormalities can recur after 6 months when the prosthetic valve is a poor match with the patient.<sup id="cite_ref-pmid12878741_28-0" class="reference"><a href="#cite_note-pmid12878741-28"><span class="cite-bracket">[</span>28<span class="cite-bracket">]</span></a></sup> Similarly, acquired VWD contributes to the bleeding tendency in people with an implant of a left <a href="/wiki/Ventricular_assist_device" title="Ventricular assist device">ventricular assist device</a> (a pump that pumps blood from the left ventricle of the heart into the aorta).<sup id="cite_ref-29" class="reference"><a href="#cite_note-29"><span class="cite-bracket">[</span>29<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Treatment">Treatment</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=17" title="Edit section: Treatment"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>For patients with VWD type 1 and VWD type 2A, <a href="/wiki/Desmopressin" title="Desmopressin">desmopressin</a> is available as different preparations, recommended for use in cases of minor trauma, or in preparation for dental or minor surgical procedures. Desmopressin stimulates the release of VWF from the <a href="/wiki/Weibel%E2%80%93Palade_body" title="Weibel–Palade body">Weibel–Palade bodies</a> of <a href="/wiki/Endothelium" title="Endothelium">endothelial cells</a>, thereby increasing the levels of VWF (as well as coagulant <a href="/wiki/Factor_VIII" title="Factor VIII">factor VIII</a>) three- to five-fold. Desmopressin is also available as a preparation for intranasal administration (Stimate) and as a preparation for intravenous administration. Desmopressin is contraindicated in VWD type 2b because of the risk of aggravated thrombocytopenia and thrombotic complications. Desmopressin is probably not effective in VWD type 2M and is rarely effective in VWD type 2N. It is totally ineffective in VWD type 3.<sup id="cite_ref-Desmopressin_24-1" class="reference"><a href="#cite_note-Desmopressin-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup> </p><p>For women with heavy menstrual bleeding, estrogen-containing oral contraceptive medications are effective in reducing the frequency and duration of the menstrual periods. Estrogen and progesterone compounds available for use in the correction of menorrhagia include <a href="/wiki/Ethinylestradiol" title="Ethinylestradiol">ethinylestradiol</a>,<a href="/wiki/Levonorgestrel" title="Levonorgestrel">levonorgestrel</a>, <a href="/wiki/Drospirenone" title="Drospirenone">drospirenone</a> and cyproterone. Administration of ethinylestradiol diminishes the secretion of luteinizing hormone and follicle-stimulating hormone from the pituitary, leading to stabilization of the endometrial surface of the uterus.<sup id="cite_ref-30" class="reference"><a href="#cite_note-30"><span class="cite-bracket">[</span>30<span class="cite-bracket">]</span></a></sup> </p><p>Desmopressin is a synthetic analog of the natural antidiuretic hormone <a href="/wiki/Vasopressin" title="Vasopressin">vasopressin</a>. Its overuse can lead to water retention and dilutional hyponatremia with consequent convulsion.<sup id="cite_ref-31" class="reference"><a href="#cite_note-31"><span class="cite-bracket">[</span>31<span class="cite-bracket">]</span></a></sup> </p><p>For patients with VWD scheduled for surgery and cases of VWD disease complicated by clinically significant hemorrhage, human-derived medium purity factor VIII concentrates, which also contain von Willebrand factors, are available for prophylaxis and treatment. Humate P, Alphanate, Wilate and Koate HP are commercially available for prophylaxis and treatment of VWD, and have varying levels of factor VIII. Products with higher VWF:RCo/FVIII ratios allow for more frequent dosing of VWF if needed, without the risk of accumulation to supranormal levels of FVIII. Recombinant factor VIII products contain insignificant quantity of VWF, so are not clinically useful as standalone therapy for VWD.<sup id="cite_ref-Connell21_8-1" class="reference"><a href="#cite_note-Connell21-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> </p><p>Risks of thrombosis, development of alloantibodies, and allergic reactions including anaphylaxis must be considered when administering these preparations. Such risks have emerged as the main concerns in factor replacement therapies as infectious risks have diminished.<sup id="cite_ref-32" class="reference"><a href="#cite_note-32"><span class="cite-bracket">[</span>32<span class="cite-bracket">]</span></a></sup> </p><p>Blood transfusions are given as needed to correct anemia and hypotension secondary to hypovolemia. Infusion of platelet concentrates is recommended for correction of hemorrhage associated with platelet-type VWD.<sup id="cite_ref-33" class="reference"><a href="#cite_note-33"><span class="cite-bracket">[</span>33<span class="cite-bracket">]</span></a></sup> </p><p><a href="/wiki/Vonicog_alfa" title="Vonicog alfa">Vonicog alfa</a> is a <a href="/wiki/Recombinant_protein" class="mw-redirect" title="Recombinant protein">recombinant</a> <a href="/wiki/Von_Willebrand_factor" title="Von Willebrand factor">von Willebrand factor</a> that was approved for use in the United States in December 2015, and for use in the European Union in August 2018.<sup id="cite_ref-Vonvendi_FDA_label_34-0" class="reference"><a href="#cite_note-Vonvendi_FDA_label-34"><span class="cite-bracket">[</span>34<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Veyvondi_EPAR_35-0" class="reference"><a href="#cite_note-Veyvondi_EPAR-35"><span class="cite-bracket">[</span>35<span class="cite-bracket">]</span></a></sup> If baseline factor VIII activity is >40%, rVWF may be administered as a standalone product when immediate response is needed, but if Factor VIII activity is <40% and immediate response is needed, rVWF must be administered in conjunction with FVIII replacement therapy.<sup id="cite_ref-36" class="reference"><a href="#cite_note-36"><span class="cite-bracket">[</span>36<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Epidemiology">Epidemiology</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=18" title="Edit section: Epidemiology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The <a href="/wiki/Prevalence" title="Prevalence">prevalence</a> of VWD is about one in 100 individuals.<sup id="cite_ref-Haematologica_37-0" class="reference"><a href="#cite_note-Haematologica-37"><span class="cite-bracket">[</span>37<span class="cite-bracket">]</span></a></sup> However, the majority of these people do not have symptoms. The prevalence of clinically significant cases is one per 10,000.<sup id="cite_ref-Haematologica_37-1" class="reference"><a href="#cite_note-Haematologica-37"><span class="cite-bracket">[</span>37<span class="cite-bracket">]</span></a></sup> Because most forms are rather mild, they are detected more often in women, whose bleeding tendency shows during <a href="/wiki/Menstruation" title="Menstruation">menstruation</a>. It may be more severe or apparent in people with <a href="/wiki/Blood_type" title="Blood type">blood type</a> O.<sup id="cite_ref-Rodak's_Hematology:_Clinical_Principles_and_Applications_38-0" class="reference"><a href="#cite_note-Rodak's_Hematology:_Clinical_Principles_and_Applications-38"><span class="cite-bracket">[</span>38<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="History">History</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=19" title="Edit section: History"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236090951"><div role="note" class="hatnote navigation-not-searchable">See also: <a href="/wiki/Erik_Adolf_von_Willebrand#Von_Willebrand_disease" title="Erik Adolf von Willebrand">Erik Adolf von Willebrand § Von Willebrand disease</a></div> <p>In 1924, a 5-year-old girl from <a href="/wiki/F%C3%B6gl%C3%B6" title="Föglö">Föglö</a>, <a href="/wiki/%C3%85land" title="Åland">Åland</a>, Finland, was brought to the Deaconess Hospital in Helsinki, where she was seen by Finnish physician <a href="/wiki/Erik_Adolf_von_Willebrand" title="Erik Adolf von Willebrand">Erik Adolf von Willebrand</a>. He ultimately assessed 66 members of her family and reported in a 1926 Swedish-language article that this was a previously undescribed bleeding disorder that differed from hemophilia.<sup id="cite_ref-pmid10444294_39-0" class="reference"><a href="#cite_note-pmid10444294-39"><span class="cite-bracket">[</span>39<span class="cite-bracket">]</span></a></sup> He published another article on the disorder in 1931, in the German language, which attracted international attention in the disease.<sup id="cite_ref-40" class="reference"><a href="#cite_note-40"><span class="cite-bracket">[</span>40<span class="cite-bracket">]</span></a></sup> The eponymous name was assigned to the disease between the late 1930s and the early 1940s, in recognition of von Willebrand's extensive research.<sup id="cite_ref-41" class="reference"><a href="#cite_note-41"><span class="cite-bracket">[</span>41<span class="cite-bracket">]</span></a></sup> </p><p>In the 1950s, it became clear that a "plasma factor", <a href="/wiki/Factor_VIII" title="Factor VIII">factor VIII</a>, was decreased in these persons and that <a href="/wiki/Cohn_process#Process_details" title="Cohn process">Cohn fraction I-0</a> could correct both the plasma deficiency of FVIII and the prolonged bleeding time. Since this time, the factor causing the long bleeding time was called the "von Willebrand factor" in honor of Erik Adolf von Willebrand. </p><p>Variant forms of VWF were recognized in the 1970s, and these variations are now recognized as the result of synthesis of an abnormal protein. During the 1980s, molecular and cellular studies distinguished hemophilia A and VWD more precisely. Persons who had VWD had a normal FVIII gene on the X chromosome, and some had an abnormal VWF gene on chromosome 12. Gene sequencing identified many of these persons as having a VWF gene mutation. The genetic causes of milder forms of low VWF are still under investigation, and these forms may not always be caused by an abnormal VWF gene. </p> <div class="mw-heading mw-heading2"><h2 id="Other_animals">Other animals</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=20" title="Edit section: Other animals"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>VWD can also affect dogs, pigs, and mice. Furthermore, cases have been reported in cats, horses, cattle, and rabbits. </p><p>The causal mutation for VWD type 1 was identified in dogs of the breeds <a href="/wiki/Doberman_Pinscher" class="mw-redirect" title="Doberman Pinscher">Doberman Pinscher</a>, <a href="/wiki/German_Pinscher" title="German Pinscher">German Pinscher</a>, <a href="/wiki/Bernese_Mountain_Dog" title="Bernese Mountain Dog">Bernese Mountain Dog</a>, <a href="/wiki/Manchester_Terrier" title="Manchester Terrier">Manchester Terrier</a>, <a href="/wiki/Kerry_Blue_Terrier" title="Kerry Blue Terrier">Kerry Blue Terrier</a>, <a href="/wiki/Cardigan_Welsh_Corgi" title="Cardigan Welsh Corgi">Cardigan Welsh Corgi</a>, <a href="/wiki/Poodle" title="Poodle">Poodle</a>, <a href="/wiki/Coton_de_Tulear" title="Coton de Tulear">Coton de Tulear</a>, <a href="/wiki/Drentse_Patrijshond" title="Drentse Patrijshond">Drentse Patrijshond</a>, <a href="/wiki/Papillon_(dog)" class="mw-redirect" title="Papillon (dog)">Papillon</a>, and <a href="/wiki/Stabyhoun" title="Stabyhoun">Stabyhoun</a>. Causal mutations for type 2 were identified in dogs of the breeds <a href="/wiki/German_Wirehaired_Pointer" title="German Wirehaired Pointer">German Wirehaired Pointer</a>, <a href="/wiki/German_Shorthaired_Pointer" title="German Shorthaired Pointer">German Shorthaired Pointer</a>, and <a href="/wiki/Chinese_Crested" class="mw-redirect" title="Chinese Crested">Chinese Crested</a>; and for type 3 in dogs of the breeds <a href="/wiki/Kooikerhondje" title="Kooikerhondje">Kooikerhondje</a>, <a href="/wiki/Scottish_Terrier" title="Scottish Terrier">Scottish Terrier</a> and <a href="/wiki/Shetland_Sheepdog" title="Shetland Sheepdog">Shetland Sheepdog</a>. In dogs affected by type 1 VWD, the causal mutation was the same across all breeds and the same mutation was also detected in some human VWD type 1 patients. In contrast, the mutations causing VWD type 3 in dogs are specific to each breed. Genetic screening is offered for known breeds.<sup id="cite_ref-42" class="reference"><a href="#cite_note-42"><span class="cite-bracket">[</span>42<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-43" class="reference"><a href="#cite_note-43"><span class="cite-bracket">[</span>43<span class="cite-bracket">]</span></a></sup> </p><p>In pigs, the causal mutation for VWD type 3 has also been identified. It is a large duplication within the VWF gene and causes serious damage to the gene function, so that virtually no VWF protein is produced. The clinical picture in pigs is most similar to that in humans with VWD type 3. Therefore, those pigs are valuable models for clinical and pharmacological research.<sup id="cite_ref-44" class="reference"><a href="#cite_note-44"><span class="cite-bracket">[</span>44<span class="cite-bracket">]</span></a></sup> </p><p>Mice affected by VWD type 3 were produced by genetic engineering to obtain a small sized model for the human disease. In these strains, the VWF gene has been knocked out.<sup id="cite_ref-45" class="reference"><a href="#cite_note-45"><span class="cite-bracket">[</span>45<span class="cite-bracket">]</span></a></sup> </p><p>In animals of other species affected by VWD, the causal mutations have not yet been identified. </p> <div class="mw-heading mw-heading2"><h2 id="Oral_manifestations">Oral manifestations</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=21" title="Edit section: Oral manifestations"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>In the case of severe deficiency, there may be spontaneous gingival bleeding, <a href="/wiki/Ecchymosis" class="mw-redirect" title="Ecchymosis">ecchymosis</a>, and <a href="/wiki/Nosebleed" title="Nosebleed">epistaxis</a>. Symptoms of VWD include postoperative bleeding, bleeding after dental extraction, gingival bleeding, epistaxis and easy bruising. The intake of oral contraceptives as the first-line treatment for <a href="/wiki/Heavy_menstrual_bleeding" title="Heavy menstrual bleeding">menorrhagia</a> may lead to gingival enlargement and bleeding in women.<sup id="cite_ref-46" class="reference"><a href="#cite_note-46"><span class="cite-bracket">[</span>46<span class="cite-bracket">]</span></a></sup> </p><p>Platelet or coagulation disorders with severely altered hemostasis can cause spontaneous gingival bleeding, as seen in conjunction with hyperplastic hyperemic gingival enlargements in leukemic patients. Deposition of <a href="/wiki/Hemosiderin" title="Hemosiderin">hemosiderin</a> and other blood degradation products on the tooth surfaces turning them brown can occur with continuous oral bleeding over long periods.<sup id="cite_ref-Glick15_47-0" class="reference"><a href="#cite_note-Glick15-47"><span class="cite-bracket">[</span>47<span class="cite-bracket">]</span></a></sup> </p><p>The location of oral bleeds was as follows: labial frenum, 60%; tongue, 23%; buccal mucosa, 17% and gingiva and palate, 0.5%. Severe hemophilia will have most frequent bleeding occurrences, followed by moderate and then mild hemophilia. They mostly come from traumatic injuries. Bleeding will also be induced by iatrogenic factors and poor oral hygiene practices. The frequency of oral hemorrhage by location in people with deficiency of F VIII and F IX is: gingiva, 64%; dental pulp, 13%; tongue, 7.5%; lip, 7%; palate, 2% and buccal mucosa, 1%.<sup id="cite_ref-Glick15_47-1" class="reference"><a href="#cite_note-Glick15-47"><span class="cite-bracket">[</span>47<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Dental_considerations">Dental considerations</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=22" title="Edit section: Dental considerations"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The protocols suggest the use of factor concentrate along with the use of local hemostatic techniques, such as suturing, and local measures, such as the use of oxidized cellulose, for example, Surgicel or fibrin glue in conjunction with post-operatively administered antifibrinolytic agents where appropriate.<sup id="cite_ref-:1_48-0" class="reference"><a href="#cite_note-:1-48"><span class="cite-bracket">[</span>48<span class="cite-bracket">]</span></a></sup> </p><p>The use of any non-steroidal anti-inflammatory drug (NSAID) must be discussed beforehand with the patient's hematologist because of their effect on platelet aggregation. There are no restrictions regarding the type of local anaesthetic agent used although those with vasoconstrictors may provide additional local hemostasis.<sup id="cite_ref-:1_48-1" class="reference"><a href="#cite_note-:1-48"><span class="cite-bracket">[</span>48<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="See_also">See also</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=23" title="Edit section: See also"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <ul><li><a href="/wiki/Bernard%E2%80%93Soulier_syndrome" title="Bernard–Soulier syndrome">Bernard–Soulier syndrome</a>, caused by a deficiency in the VWF receptor, <a href="/wiki/Glycoprotein_Ib" title="Glycoprotein Ib">GPIb</a></li> <li><a href="/wiki/List_of_hematologic_conditions" title="List of hematologic conditions">List of hematologic conditions</a></li> <li><a href="/wiki/Purpura" title="Purpura">Purpura</a></li></ul> <div class="mw-heading mw-heading2"><h2 id="References">References</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=24" title="Edit section: References"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1239543626">.mw-parser-output .reflist{margin-bottom:0.5em;list-style-type:decimal}@media screen{.mw-parser-output .reflist{font-size:90%}}.mw-parser-output .reflist .references{font-size:100%;margin-bottom:0;list-style-type:inherit}.mw-parser-output .reflist-columns-2{column-width:30em}.mw-parser-output .reflist-columns-3{column-width:25em}.mw-parser-output .reflist-columns{margin-top:0.3em}.mw-parser-output .reflist-columns ol{margin-top:0}.mw-parser-output .reflist-columns li{page-break-inside:avoid;break-inside:avoid-column}.mw-parser-output .reflist-upper-alpha{list-style-type:upper-alpha}.mw-parser-output .reflist-upper-roman{list-style-type:upper-roman}.mw-parser-output .reflist-lower-alpha{list-style-type:lower-alpha}.mw-parser-output .reflist-lower-greek{list-style-type:lower-greek}.mw-parser-output .reflist-lower-roman{list-style-type:lower-roman}</style><div class="reflist"> <div class="mw-references-wrap mw-references-columns"><ol class="references"> <li id="cite_note-1"><span class="mw-cite-backlink"><b><a href="#cite_ref-1">^</a></b></span> <span class="reference-text"><i><a href="/wiki/MedlinePlus" title="MedlinePlus">MedlinePlus Encyclopedia</a></i>: <a rel="nofollow" class="external text" href="https://medlineplus.gov/ency/article/000544.htm">Von Willebrand disease</a></span> </li> <li id="cite_note-Kaur24-2"><span class="mw-cite-backlink">^ <a href="#cite_ref-Kaur24_2-0"><sup><i><b>a</b></i></sup></a> <a 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This topic last updated: Feb 05, 2021. In turn adapted from The National Heart, Lung and Blood Institute. The Diagnosis, Evaluation, and management of Von Willebrand Disease. National Institutes of Health Publication 08-5832, December 2007</span> </li> <li id="cite_note-27"><span class="mw-cite-backlink"><b><a href="#cite_ref-27">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFTiedeRandBuddeGanser2011" class="citation journal cs1">Tiede A, Rand JH, Budde U, Ganser A, Federici AB (June 2011). 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class="external text" href="https://www.vetgen.com/canine-VWD.html">"Canine von Willebrand Disease"</a>. <i>vetgen.com</i>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=vetgen.com&rft.atitle=Canine+von+Willebrand+Disease&rft_id=https%3A%2F%2Fwww.vetgen.com%2Fcanine-VWD.html&rfr_id=info%3Asid%2Fen.wikipedia.org%3AVon+Willebrand+disease" class="Z3988"></span></span> </li> <li id="cite_note-44"><span class="mw-cite-backlink"><b><a href="#cite_ref-44">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFLehnerEkhlasi-HundrieserDeteringAllerkamp2018" class="citation journal cs1">Lehner S, Ekhlasi-Hundrieser M, Detering C, Allerkamp H, Pfarrer C, von Depka Prondzinski M (February 2018). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5919753">"VWF Gene in Pigs Affected by Von Willebrand Disease Type 3"</a>. <i>G3</i>. <b>8</b> (2): 577–585. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1534%2Fg3.117.300432">10.1534/g3.117.300432</a>. <a href="/wiki/PMC_(identifier)" class="mw-redirect" title="PMC (identifier)">PMC</a> <span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5919753">5919753</a></span>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/29208651">29208651</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=G3&rft.atitle=VWF+Gene+in+Pigs+Affected+by+Von+Willebrand+Disease+Type+3&rft.volume=8&rft.issue=2&rft.pages=577-585&rft.date=2018-02&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC5919753%23id-name%3DPMC&rft_id=info%3Apmid%2F29208651&rft_id=info%3Adoi%2F10.1534%2Fg3.117.300432&rft.aulast=Lehner&rft.aufirst=S&rft.au=Ekhlasi-Hundrieser%2C+M&rft.au=Detering%2C+C&rft.au=Allerkamp%2C+H&rft.au=Pfarrer%2C+C&rft.au=von+Depka+Prondzinski%2C+M&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC5919753&rfr_id=info%3Asid%2Fen.wikipedia.org%3AVon+Willebrand+disease" class="Z3988"></span></span> </li> <li id="cite_note-45"><span class="mw-cite-backlink"><b><a href="#cite_ref-45">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFLozierNichols2013" class="citation journal cs1">Lozier JN, Nichols TC (April 2013). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3742033">"Animal models of hemophilia and related bleeding disorders"</a>. <i>Seminars in Hematology</i>. <b>50</b> (2): 175–84. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1053%2Fj.seminhematol.2013.03.023">10.1053/j.seminhematol.2013.03.023</a>. <a href="/wiki/PMC_(identifier)" class="mw-redirect" title="PMC (identifier)">PMC</a> <span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3742033">3742033</a></span>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/23956467">23956467</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Seminars+in+Hematology&rft.atitle=Animal+models+of+hemophilia+and+related+bleeding+disorders&rft.volume=50&rft.issue=2&rft.pages=175-84&rft.date=2013-04&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC3742033%23id-name%3DPMC&rft_id=info%3Apmid%2F23956467&rft_id=info%3Adoi%2F10.1053%2Fj.seminhematol.2013.03.023&rft.aulast=Lozier&rft.aufirst=JN&rft.au=Nichols%2C+TC&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC3742033&rfr_id=info%3Asid%2Fen.wikipedia.org%3AVon+Willebrand+disease" class="Z3988"></span></span> </li> <li id="cite_note-46"><span class="mw-cite-backlink"><b><a href="#cite_ref-46">^</a></b></span> <span class="reference-text"><i>"Hemophilia A" (PDF). College of Dental Hygienists of Ontario. September 2, 2015.</i></span> </li> <li id="cite_note-Glick15-47"><span class="mw-cite-backlink">^ <a href="#cite_ref-Glick15_47-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-Glick15_47-1"><sup><i><b>b</b></i></sup></a></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFGlick2015" class="citation book cs1">Glick, Michael (2015). <i>Burket's Oral Medicine</i>. People's Medical. pp. 473, 475, 481, 482. <a href="/wiki/ISBN_(identifier)" class="mw-redirect" title="ISBN (identifier)">ISBN</a> <a href="/wiki/Special:BookSources/978-1-60795-188-9" title="Special:BookSources/978-1-60795-188-9"><bdi>978-1-60795-188-9</bdi></a>. <a href="/wiki/OCLC_(identifier)" class="mw-redirect" title="OCLC (identifier)">OCLC</a> <a rel="nofollow" class="external text" href="https://search.worldcat.org/oclc/903962852">903962852</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=book&rft.btitle=Burket%27s+Oral+Medicine&rft.pages=473%2C+475%2C+481%2C+482&rft.pub=People%27s+Medical&rft.date=2015&rft_id=info%3Aoclcnum%2F903962852&rft.isbn=978-1-60795-188-9&rft.aulast=Glick&rft.aufirst=Michael&rfr_id=info%3Asid%2Fen.wikipedia.org%3AVon+Willebrand+disease" class="Z3988"></span></span> </li> <li id="cite_note-:1-48"><span class="mw-cite-backlink">^ <a href="#cite_ref-:1_48-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-:1_48-1"><sup><i><b>b</b></i></sup></a></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFBrewerCorrea2006" class="citation techreport cs1">Brewer, Andrew; Correa, Maria Elvira (May 2006). <a rel="nofollow" class="external text" href="https://www1.wfh.org/publication/files/pdf-1190.pdf"><i>Guidelines for dental treatment of patients with inherited bleeding disorders</i></a> <span class="cs1-format">(PDF)</span> (Monograph). <a href="/wiki/World_Federation_of_Hemophilia" title="World Federation of Hemophilia">World Federation of Hemophilia</a>. 40.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=report&rft.btitle=Guidelines+for+dental+treatment+of+patients+with+inherited+bleeding+disorders&rft.pub=World+Federation+of+Hemophilia&rft.date=2006-05&rft.aulast=Brewer&rft.aufirst=Andrew&rft.au=Correa%2C+Maria+Elvira&rft_id=https%3A%2F%2Fwww1.wfh.org%2Fpublication%2Ffiles%2Fpdf-1190.pdf&rfr_id=info%3Asid%2Fen.wikipedia.org%3AVon+Willebrand+disease" class="Z3988"></span></span> </li> </ol></div></div> <div class="mw-heading mw-heading2"><h2 id="Further_reading">Further reading</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=25" title="Edit section: Further reading"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1239549316">.mw-parser-output .refbegin{margin-bottom:0.5em}.mw-parser-output .refbegin-hanging-indents>ul{margin-left:0}.mw-parser-output .refbegin-hanging-indents>ul>li{margin-left:0;padding-left:3.2em;text-indent:-3.2em}.mw-parser-output .refbegin-hanging-indents ul,.mw-parser-output .refbegin-hanging-indents ul li{list-style:none}@media(max-width:720px){.mw-parser-output .refbegin-hanging-indents>ul>li{padding-left:1.6em;text-indent:-1.6em}}.mw-parser-output .refbegin-columns{margin-top:0.3em}.mw-parser-output .refbegin-columns ul{margin-top:0}.mw-parser-output .refbegin-columns li{page-break-inside:avoid;break-inside:avoid-column}@media screen{.mw-parser-output .refbegin{font-size:90%}}</style><div class="refbegin" style=""> <ul><li><i>Harrison's textbook of Internal Medicine</i>, Chapter 177.</li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFSadler1998" class="citation journal cs1">Sadler JE (1998). <a rel="nofollow" class="external text" href="https://doi.org/10.1146%2Fannurev.biochem.67.1.395">"Biochemistry and genetics of von Willebrand factor"</a>. <i>Annual Review of Biochemistry</i>. <b>67</b>: 395–424. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://doi.org/10.1146%2Fannurev.biochem.67.1.395">10.1146/annurev.biochem.67.1.395</a></span>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/9759493">9759493</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Annual+Review+of+Biochemistry&rft.atitle=Biochemistry+and+genetics+of+von+Willebrand+factor&rft.volume=67&rft.pages=395-424&rft.date=1998&rft_id=info%3Adoi%2F10.1146%2Fannurev.biochem.67.1.395&rft_id=info%3Apmid%2F9759493&rft.aulast=Sadler&rft.aufirst=JE&rft_id=https%3A%2F%2Fdoi.org%2F10.1146%252Fannurev.biochem.67.1.395&rfr_id=info%3Asid%2Fen.wikipedia.org%3AVon+Willebrand+disease" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFMannucci2004" class="citation journal cs1">Mannucci PM (August 2004). "Treatment of von Willebrand's Disease". <i>The New England Journal of Medicine</i>. <b>351</b> (7): 683–94. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1056%2FNEJMra040403">10.1056/NEJMra040403</a>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/15306670">15306670</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=The+New+England+Journal+of+Medicine&rft.atitle=Treatment+of+von+Willebrand%27s+Disease&rft.volume=351&rft.issue=7&rft.pages=683-94&rft.date=2004-08&rft_id=info%3Adoi%2F10.1056%2FNEJMra040403&rft_id=info%3Apmid%2F15306670&rft.aulast=Mannucci&rft.aufirst=PM&rfr_id=info%3Asid%2Fen.wikipedia.org%3AVon+Willebrand+disease" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFLaffanBrownCollinsCumming2004" class="citation journal cs1">Laffan M, Brown SA, Collins PW, Cumming AM, Hill FG, Keeling D, Peake IR, Pasi KJ (May 2004). "The diagnosis of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization". <i>Haemophilia</i>. <b>10</b> (3): 199–217. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1111%2Fj.1365-2516.2004.00894.x">10.1111/j.1365-2516.2004.00894.x</a>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/15086318">15086318</a>. <a href="/wiki/S2CID_(identifier)" class="mw-redirect" title="S2CID (identifier)">S2CID</a> <a rel="nofollow" class="external text" href="https://api.semanticscholar.org/CorpusID:731315">731315</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Haemophilia&rft.atitle=The+diagnosis+of+von+Willebrand+disease%3A+a+guideline+from+the+UK+Haemophilia+Centre+Doctors%27+Organization&rft.volume=10&rft.issue=3&rft.pages=199-217&rft.date=2004-05&rft_id=https%3A%2F%2Fapi.semanticscholar.org%2FCorpusID%3A731315%23id-name%3DS2CID&rft_id=info%3Apmid%2F15086318&rft_id=info%3Adoi%2F10.1111%2Fj.1365-2516.2004.00894.x&rft.aulast=Laffan&rft.aufirst=M&rft.au=Brown%2C+SA&rft.au=Collins%2C+PW&rft.au=Cumming%2C+AM&rft.au=Hill%2C+FG&rft.au=Keeling%2C+D&rft.au=Peake%2C+IR&rft.au=Pasi%2C+KJ&rfr_id=info%3Asid%2Fen.wikipedia.org%3AVon+Willebrand+disease" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFLichtmanWilliams2006" class="citation book cs1">Lichtman MA, Williams WJ (2006). <i>Williams Hematology</i> (7th ed.). McGraw-Hill. <a href="/wiki/ISBN_(identifier)" class="mw-redirect" title="ISBN (identifier)">ISBN</a> <a href="/wiki/Special:BookSources/978-0-07-143591-8" title="Special:BookSources/978-0-07-143591-8"><bdi>978-0-07-143591-8</bdi></a>. <a href="/wiki/OCLC_(identifier)" class="mw-redirect" title="OCLC (identifier)">OCLC</a> <a rel="nofollow" class="external text" href="https://search.worldcat.org/oclc/780625008">780625008</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=book&rft.btitle=Williams+Hematology&rft.edition=7th&rft.pub=McGraw-Hill&rft.date=2006&rft_id=info%3Aoclcnum%2F780625008&rft.isbn=978-0-07-143591-8&rft.aulast=Lichtman&rft.aufirst=MA&rft.au=Williams%2C+WJ&rfr_id=info%3Asid%2Fen.wikipedia.org%3AVon+Willebrand+disease" class="Z3988"></span> <ul><li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite class="citation book cs1"><i>Ch. 112 Hereditary qualitative platelet disorders</i>. pp. 1806–.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=book&rft.btitle=Ch.+112+Hereditary+qualitative+platelet+disorders&rft.pages=1806-&rfr_id=info%3Asid%2Fen.wikipedia.org%3AVon+Willebrand+disease" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite class="citation book cs1"><i>Ch. 118 von Willebrand disease</i>. pp. 1937–.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=book&rft.btitle=Ch.+118+von+Willebrand+disease&rft.pages=1937-&rfr_id=info%3Asid%2Fen.wikipedia.org%3AVon+Willebrand+disease" class="Z3988"></span></li></ul></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFGoodeveJames2017" class="citation book cs1">Goodeve A, James P (October 2017). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/books/NBK7014/"><i>von Willebrand Disease</i></a>. GeneReviews® [Internet]. University of Washington, Seattle. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/20301765">20301765</a>. NBK7014.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=book&rft.btitle=von+Willebrand+Disease&rft.series=GeneReviews%C2%AE+%5BInternet%5D&rft.pub=University+of+Washington%2C+Seattle&rft.date=2017-10&rft_id=info%3Apmid%2F20301765&rft.aulast=Goodeve&rft.aufirst=A&rft.au=James%2C+P&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fbooks%2FNBK7014%2F&rfr_id=info%3Asid%2Fen.wikipedia.org%3AVon+Willebrand+disease" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFNHLBI_von_Willebrand_Disease_Expert_Panel2008" class="citation report cs1">NHLBI von Willebrand Disease Expert Panel (January 2008). <a rel="nofollow" class="external text" href="https://www.nhlbi.nih.gov/health-topics/diagnosis-evaluation-and-management-of-von-willebrand-disease">The Diagnosis, Evaluation and Management of von Willebrand Disease</a>. <i>National Heart, Lung, and Blood Institute</i> (Report).</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=report&rft.btitle=The+Diagnosis%2C+Evaluation+and+Management+of+von+Willebrand+Disease&rft.date=2008-01&rft.au=NHLBI+von+Willebrand+Disease+Expert+Panel&rft_id=https%3A%2F%2Fwww.nhlbi.nih.gov%2Fhealth-topics%2Fdiagnosis-evaluation-and-management-of-von-willebrand-disease&rfr_id=info%3Asid%2Fen.wikipedia.org%3AVon+Willebrand+disease" class="Z3988"></span></li></ul> </div> <div class="mw-heading mw-heading2"><h2 id="External_links">External links</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Von_Willebrand_disease&action=edit&section=26" title="Edit section: External links"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <div class="navbox-styles"><style data-mw-deduplicate="TemplateStyles:r1129693374">.mw-parser-output .hlist dl,.mw-parser-output .hlist ol,.mw-parser-output .hlist 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.navbar-collapse{float:left;text-align:left}.mw-parser-output .navbar-boxtext{word-spacing:0}.mw-parser-output .navbar ul{display:inline-block;white-space:nowrap;line-height:inherit}.mw-parser-output .navbar-brackets::before{margin-right:-0.125em;content:"[ "}.mw-parser-output .navbar-brackets::after{margin-left:-0.125em;content:" ]"}.mw-parser-output .navbar li{word-spacing:-0.125em}.mw-parser-output .navbar a>span,.mw-parser-output .navbar a>abbr{text-decoration:inherit}.mw-parser-output .navbar-mini abbr{font-variant:small-caps;border-bottom:none;text-decoration:none;cursor:inherit}.mw-parser-output .navbar-ct-full{font-size:114%;margin:0 7em}.mw-parser-output .navbar-ct-mini{font-size:114%;margin:0 4em}html.skin-theme-clientpref-night .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}@media(prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}}@media print{.mw-parser-output .navbar{display:none!important}}</style><div class="navbar plainlinks hlist navbar-mini"><ul><li class="nv-view"><a href="/wiki/Template:Bleeding_and_clotting_disorders" title="Template:Bleeding and clotting disorders"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Bleeding_and_clotting_disorders" title="Template talk:Bleeding and clotting disorders"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Bleeding_and_clotting_disorders" title="Special:EditPage/Template:Bleeding and clotting disorders"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Disorders_of_bleeding_and_clotting" style="font-size:114%;margin:0 4em">Disorders of <a href="/wiki/Bleeding" title="Bleeding">bleeding</a> and <a href="/wiki/Coagulation" title="Coagulation">clotting</a></div></th></tr><tr><td class="navbox-abovebelow hlist" colspan="2"><div> <ul><li><a href="/wiki/Coagulation" title="Coagulation">Coagulation</a></li> <li><a href="/wiki/Coagulopathy" title="Coagulopathy">coagulopathy</a></li> <li><a href="/wiki/Bleeding_diathesis" title="Bleeding diathesis">Bleeding diathesis</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Clotting</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%">By cause</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Clotting_factors" class="mw-redirect" title="Clotting factors">Clotting factors</a> <ul><li><a href="/wiki/Antithrombin_III_deficiency" title="Antithrombin III deficiency">Antithrombin III deficiency</a></li> <li><a href="/wiki/Protein_C_deficiency" title="Protein C deficiency">Protein C deficiency</a></li> <li><a href="/wiki/Activated_protein_C_resistance" title="Activated protein C resistance">Activated protein C resistance</a></li> <li><a href="/wiki/Protein_S_deficiency" title="Protein S deficiency">Protein S deficiency</a></li> <li><a href="/wiki/Factor_V_Leiden" title="Factor V Leiden">Factor V Leiden</a></li> <li><a href="/wiki/Prothrombin_G20210A" title="Prothrombin G20210A">Prothrombin G20210A</a></li></ul></li> <li><a href="/wiki/Platelets" class="mw-redirect" title="Platelets">Platelets</a> <ul><li><a href="/wiki/Sticky_platelet_syndrome" title="Sticky platelet syndrome">Sticky platelet syndrome</a></li> <li><a href="/wiki/Thrombocytosis" class="mw-redirect" title="Thrombocytosis">Thrombocytosis</a></li> <li><a href="/wiki/Essential_thrombocythemia" title="Essential thrombocythemia">Essential thrombocythemia</a></li></ul></li> <li><a href="/wiki/Disseminated_intravascular_coagulation" title="Disseminated intravascular coagulation">DIC</a> <ul><li><a href="/wiki/Purpura_fulminans" title="Purpura fulminans">Purpura fulminans</a></li></ul></li> <li><a href="/wiki/Antiphospholipid_syndrome" title="Antiphospholipid syndrome">Antiphospholipid syndrome</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Thrombosis" title="Thrombosis">Clots</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Thrombophilia" title="Thrombophilia">Thrombophilia</a></li> <li><a href="/wiki/Thrombus" title="Thrombus">Thrombus</a></li> <li><a href="/wiki/Thrombosis" title="Thrombosis">Thrombosis</a></li> <li><a href="/wiki/Virchow%27s_triad" title="Virchow's triad">Virchow's triad</a></li> <li><a href="/wiki/Trousseau_sign_of_malignancy" title="Trousseau sign of malignancy">Trousseau sign of malignancy</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">By site</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Deep_vein_thrombosis" title="Deep vein thrombosis">Deep vein thrombosis</a> <ul><li><a href="/wiki/Bancroft%27s_sign" title="Bancroft's sign">Bancroft's sign</a></li> <li><a href="/wiki/Homans_sign" title="Homans sign">Homans sign</a></li> <li><a href="/wiki/Lisker%27s_sign" title="Lisker's sign">Lisker's sign</a></li> <li><a href="/wiki/Louvel%27s_sign" title="Louvel's sign">Louvel's sign</a></li> <li><a href="/wiki/Lowenberg%27s_sign" title="Lowenberg's sign">Lowenberg's sign</a></li> <li><a href="/wiki/Peabody%27s_sign" title="Peabody's sign">Peabody's sign</a></li> <li><a href="/wiki/Pratt%27s_sign" title="Pratt's sign">Pratt's sign</a></li> <li><a href="/wiki/Rose%27s_sign" title="Rose's sign">Rose's sign</a></li></ul></li> <li><a href="/wiki/Pulmonary_embolism" title="Pulmonary embolism">Pulmonary embolism</a></li> <li><a href="/wiki/Renal_vein_thrombosis" title="Renal vein thrombosis">Renal vein thrombosis</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Bleeding</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th id="By_cause" scope="row" class="navbox-group" style="width:1%">By cause</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Thrombocytopenia" title="Thrombocytopenia">Thrombocytopenia</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Thrombocytopenic_purpura" title="Thrombocytopenic purpura">Thrombocytopenic purpura</a>: <a href="/wiki/Idiopathic_thrombocytopenic_purpura" class="mw-redirect" title="Idiopathic thrombocytopenic purpura">ITP</a> <ul><li><a href="/wiki/Evans_syndrome" title="Evans syndrome">Evans syndrome</a></li></ul></li> <li><a href="/wiki/Thrombotic_microangiopathy" title="Thrombotic microangiopathy">TM</a> <ul><li><a href="/wiki/Thrombotic_thrombocytopenic_purpura" title="Thrombotic thrombocytopenic purpura">TTP</a></li> <li><a href="/wiki/Upshaw%E2%80%93Schulman_syndrome" title="Upshaw–Schulman syndrome">Upshaw–Schulman syndrome</a></li></ul></li></ul> <ul><li><a href="/wiki/Heparin-induced_thrombocytopenia" title="Heparin-induced thrombocytopenia">Heparin-induced thrombocytopenia</a></li> <li><a href="/wiki/May%E2%80%93Hegglin_anomaly" title="May–Hegglin anomaly">May–Hegglin anomaly</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Platelet#Diseases" title="Platelet">Platelet function</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><i>adhesion</i> <ul><li><a href="/wiki/Bernard%E2%80%93Soulier_syndrome" title="Bernard–Soulier syndrome">Bernard–Soulier syndrome</a></li></ul></li> <li><i>aggregation</i> <ul><li><a href="/wiki/Glanzmann%27s_thrombasthenia" title="Glanzmann's thrombasthenia">Glanzmann's thrombasthenia</a></li></ul></li> <li><i><a href="/wiki/Platelet_storage_pool_deficiency" title="Platelet storage pool deficiency">platelet storage pool deficiency</a></i> <ul><li><a href="/wiki/Hermansky%E2%80%93Pudlak_syndrome" title="Hermansky–Pudlak syndrome">Hermansky–Pudlak syndrome</a></li> <li><a href="/wiki/Gray_platelet_syndrome" title="Gray platelet syndrome">Gray platelet syndrome</a></li></ul></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Clotting_factor" class="mw-redirect" title="Clotting factor">Clotting factor</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Haemophilia" title="Haemophilia">Hemophilia</a> <ul><li><a href="/wiki/Haemophilia_A" title="Haemophilia A">A/VIII</a></li> <li><a href="/wiki/Haemophilia_B" title="Haemophilia B">B/IX</a></li> <li><a href="/wiki/Haemophilia_C" title="Haemophilia C">C/XI</a></li></ul></li> <li><a class="mw-selflink selflink">von Willebrand disease</a></li> <li><a href="/wiki/Hypoprothrombinemia" title="Hypoprothrombinemia">Hypoprothrombinemia/II</a></li> <li><a href="/wiki/Factor_VII_deficiency" title="Factor VII deficiency">Factor VII deficiency</a></li> <li><a href="/wiki/Factor_X_deficiency" title="Factor X deficiency">Factor X deficiency</a></li> <li><a href="/wiki/Factor_XII_deficiency" title="Factor XII deficiency">Factor XII deficiency</a></li> <li><a href="/wiki/Factor_XIII_deficiency" title="Factor XIII deficiency">Factor XIII deficiency</a></li> <li><a href="/wiki/Dysfibrinogenemia" title="Dysfibrinogenemia">Dysfibrinogenemia</a></li> <li><a href="/wiki/Congenital_afibrinogenemia" title="Congenital afibrinogenemia">Congenital afibrinogenemia</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Signs and symptoms</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Bleeding" title="Bleeding">Bleeding</a></li> <li><a href="/wiki/Bruise" title="Bruise">Bruise</a></li> <li><a href="/wiki/Hematoma" title="Hematoma">Hematoma</a></li> <li><a href="/wiki/Petechia" title="Petechia">Petechia</a></li> <li><a href="/wiki/Purpura" title="Purpura">Purpura</a> <ul><li><a href="/wiki/Nonthrombocytopenic_purpura" title="Nonthrombocytopenic purpura">Nonthrombocytopenic purpura</a></li></ul></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">By site</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li>head <ul><li><a href="/wiki/Nosebleed" title="Nosebleed">Epistaxis</a></li> <li><a href="/wiki/Hemoptysis" title="Hemoptysis">Hemoptysis</a></li> <li><a href="/wiki/Intracranial_hemorrhage" title="Intracranial hemorrhage">Intracranial hemorrhage</a></li> <li><a href="/wiki/Hyphema" title="Hyphema">Hyphema</a></li> <li><a href="/wiki/Subconjunctival_bleeding" title="Subconjunctival bleeding">Subconjunctival bleeding</a></li></ul></li> <li>torso <ul><li><a href="/wiki/Hemothorax" title="Hemothorax">Hemothorax</a></li> <li><a href="/wiki/Hemopericardium" title="Hemopericardium">Hemopericardium</a></li> <li><a href="/wiki/Pulmonary_hematoma" title="Pulmonary hematoma">Pulmonary hematoma</a></li></ul></li> <li>abdomen <ul><li><a href="/wiki/Gastrointestinal_bleeding" title="Gastrointestinal bleeding">Gastrointestinal bleeding</a></li> <li><a href="/wiki/Hemobilia" class="mw-redirect" title="Hemobilia">Hemobilia</a></li> <li><a href="/wiki/Hemoperitoneum" title="Hemoperitoneum">Hemoperitoneum</a></li> <li><a href="/wiki/Hematocele" title="Hematocele">Hematocele</a></li> <li><a href="/wiki/Hematosalpinx" title="Hematosalpinx">Hematosalpinx</a></li></ul></li> <li>joint <ul><li><a href="/wiki/Hemarthrosis" title="Hemarthrosis">Hemarthrosis</a></li></ul></li></ul> </div></td></tr></tbody></table><div></div></td></tr></tbody></table></div> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"></div><div role="navigation" class="navbox" aria-label="Navbox" style="width:100%; margin:0.5em 0 0.5em 0;;padding:3px"><table class="nowraplinks navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">Classification</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"><div style="position:relative; float:right; font-size:0.8em;"><a href="https://www.wikidata.org/wiki/Q709360" class="extiw" title="d:Q709360">D</a></div><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/International_Statistical_Classification_of_Diseases_and_Related_Health_Problems" class="mw-redirect" title="International Statistical Classification of Diseases and Related Health Problems">ICD</a>-<a href="/wiki/ICD-10" title="ICD-10">10</a></b>: <a rel="nofollow" class="external text" href="https://icd.who.int/browse10/2019/en#/D68.0">D68.0</a></li><li><b><a href="/wiki/International_Statistical_Classification_of_Diseases_and_Related_Health_Problems" class="mw-redirect" title="International Statistical Classification of Diseases and Related Health Problems">ICD</a>-<a href="/wiki/List_of_ICD-9_codes" title="List of ICD-9 codes">9-CM</a></b>: <a rel="nofollow" class="external text" href="http://www.icd9data.com/getICD9Code.ashx?icd9=286.4">286.4</a></li><li><b><a href="/wiki/Online_Mendelian_Inheritance_in_Man" title="Online Mendelian Inheritance in Man">OMIM</a></b>: <a rel="nofollow" class="external text" href="https://omim.org/entry/193400">193400</a></li><li><b><a href="/wiki/Medical_Subject_Headings" title="Medical Subject Headings">MeSH</a></b>: <a rel="nofollow" class="external text" href="https://meshb.nlm.nih.gov/record/ui?ui=D014842">D014842</a></li><li><b><a href="/wiki/Diseases_Database" title="Diseases Database">DiseasesDB</a></b>: <a rel="nofollow" class="external text" href="http://www.diseasesdatabase.com/ddb14007.htm">14007</a></li></ul></div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">External resources</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/MedlinePlus" title="MedlinePlus">MedlinePlus</a></b>: <a rel="nofollow" class="external text" href="https://www.nlm.nih.gov/medlineplus/ency/article/000544.htm">000544</a></li><li><b><a href="/wiki/EMedicine" title="EMedicine">eMedicine</a></b>: <a rel="nofollow" class="external text" href="https://emedicine.medscape.com/ped/2419-overview">ped/2419</a></li><li><b><a href="/wiki/GeneReviews" title="GeneReviews">GeneReviews</a></b>: <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/books/NBK7014/">von Willebrand Disease</a></li></ul></div></div></td></tr></tbody></table></div> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link 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