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class="firstHeading mw-first-heading"><span class="mw-page-title-main">Hyper-IgM syndrome type 3</span></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. 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class="mw-redirect" title="IgG">IgG</a> → <a href="/wiki/Immunoglobulin_G" title="Immunoglobulin G">Immunoglobulin G</a>)</span></span>. The present address (URL) is a <a href="/wiki/Help:Permanent_link" title="Help:Permanent link">permanent link</a> to this revision, which may differ significantly from the <span class="plainlinks"><a class="external text" href="https://en.wikipedia.org/wiki/Hyper-IgM_syndrome_type_3">current revision</a></span>.</b></p><div id="revision-info-plain" style="display: none;">Revision as of 14:14, 14 October 2020 by <a href="/wiki/User:Onel5969" class="mw-userlink" title="User:Onel5969" data-mw-revid="983485631"><bdi>Onel5969</bdi></a> <span class="mw-usertoollinks">(<a href="/wiki/User_talk:Onel5969" class="mw-usertoollinks-talk" title="User talk:Onel5969">talk</a> | <a href="/wiki/Special:Contributions/Onel5969" class="mw-usertoollinks-contribs" title="Special:Contributions/Onel5969">contribs</a>)</span> <span class="comment">(Disambiguated: <a href="/wiki/IgG" class="mw-redirect" title="IgG">IgG</a> → <a href="/wiki/Immunoglobulin_G" title="Immunoglobulin G">Immunoglobulin G</a>)</span></div></div><div id="mw-revision-nav">(<a href="/w/index.php?title=Hyper-IgM_syndrome_type_3&diff=prev&oldid=983485631" title="Hyper-IgM syndrome type 3">diff</a>) <a href="/w/index.php?title=Hyper-IgM_syndrome_type_3&direction=prev&oldid=983485631" title="Hyper-IgM syndrome type 3">← Previous revision</a> | <a href="/wiki/Hyper-IgM_syndrome_type_3" title="Hyper-IgM syndrome type 3">Latest revision</a> (<a href="/w/index.php?title=Hyper-IgM_syndrome_type_3&diff=cur&oldid=983485631" title="Hyper-IgM syndrome type 3">diff</a>) | <a href="/w/index.php?title=Hyper-IgM_syndrome_type_3&direction=next&oldid=983485631" title="Hyper-IgM syndrome type 3">Newer revision →</a> (<a href="/w/index.php?title=Hyper-IgM_syndrome_type_3&diff=next&oldid=983485631" title="Hyper-IgM syndrome type 3">diff</a>)</div></div></div></div></div> <div id="mw-content-text" class="mw-body-content"><div class="mw-content-ltr mw-parser-output" lang="en" dir="ltr"><p><br /> </p><p><br /> </p> <div class="shortdescription nomobile noexcerpt noprint searchaux" style="display:none">Primary immune deficiency disorder characterized by defective CD40 signaling</div> <p class="mw-empty-elt"> </p> <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">Hyper IgM syndrome type 3</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:IgM_scheme.svg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/7/77/IgM_scheme.svg/220px-IgM_scheme.svg.png" decoding="async" width="220" height="211" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/7/77/IgM_scheme.svg/330px-IgM_scheme.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/7/77/IgM_scheme.svg/440px-IgM_scheme.svg.png 2x" data-file-width="400" data-file-height="384" /></a></span></td></tr><tr><td colspan="2" class="infobox-full-data"><a href="/wiki/Immunoglobulin_M" title="Immunoglobulin M">Immunoglobulin M</a></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> <span class="penicon autoconfirmed-show"><span class="mw-valign-text-top" typeof="mw:File/Frameless"><a href="https://www.wikidata.org/wiki/Q5957519?uselang=en#P1995" title="Edit this on Wikidata"><img alt="Edit this on Wikidata" src="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/10px-OOjs_UI_icon_edit-ltr-progressive.svg.png" decoding="async" width="10" height="10" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/15px-OOjs_UI_icon_edit-ltr-progressive.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/20px-OOjs_UI_icon_edit-ltr-progressive.svg.png 2x" data-file-width="20" data-file-height="20" /></a></span></span></td></tr><tr><th scope="row" class="infobox-label">Types</th><td class="infobox-data">Hyper-IgM syndrome type 1,2,3,4 and 5<sup id="cite_ref-om1_1-0" class="reference"><a href="#cite_note-om1-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-om2_2-0" class="reference"><a href="#cite_note-om2-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-om3_3-0" class="reference"><a href="#cite_note-om3-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-om5_4-0" class="reference"><a href="#cite_note-om5-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup><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></td></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Medical_diagnosis" title="Medical diagnosis">Diagnostic method</a></th><td class="infobox-data">MRI, Chest radiography and genetic testing<sup id="cite_ref-emed_6-0" class="reference"><a href="#cite_note-emed-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup></td></tr><tr><th scope="row" class="infobox-label">Treatment</th><td class="infobox-data">Allogeneic hematopoietic cell transplantation<sup id="cite_ref-gen_7-0" class="reference"><a href="#cite_note-gen-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup></td></tr></tbody></table> <p><b>Hyper-IgM syndrome type 3</b> is a form of <a href="/wiki/Hyper_IgM_syndrome" title="Hyper IgM syndrome">hyper IgM syndrome</a> characterized by mutations of the <i><a href="/wiki/CD40" class="mw-redirect" title="CD40">CD40</a></i> gene.<sup id="cite_ref-8" class="reference"><a href="#cite_note-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> In this type, Immature B cells cannot receive signal 2 from helper T cells which is necessary to mature into mature B cells. </p><p><br /> </p> <meta property="mw:PageProp/toc" /> <div class="mw-heading mw-heading2"><h2 id="Hyper_IgM_syndromes">Hyper IgM syndromes</h2></div> <p><b>Hyper IgM syndromes</b> is a group of <a href="/wiki/Primary_immunodeficiency" title="Primary immunodeficiency">primary immune deficiency disorders</a> characterized by defective <a href="/wiki/CD40" class="mw-redirect" title="CD40">CD40</a> signaling; <i>via</i> <a href="/wiki/B_cell" title="B cell">B cells</a> affecting <a href="/wiki/Immunoglobulin_class_switching" title="Immunoglobulin class switching">class switch recombination</a> (CSR) and <a href="/wiki/Somatic_hypermutation" title="Somatic hypermutation">somatic hypermutation</a>. <a href="/wiki/Immunoglobulin" class="mw-redirect" title="Immunoglobulin">Immunoglobulin</a> (Ig) class switch recombination deficiencies are characterized by elevated serum IgM levels and a considerable deficiency in <a href="/wiki/Immunoglobulin_G" title="Immunoglobulin G">Immunoglobulins G</a> (IgG), <a href="/wiki/Immunoglobulin_A" title="Immunoglobulin A">A</a> (IgA) and <a href="/wiki/Immunoglobulin_E" title="Immunoglobulin E">E</a> (IgE). As a consequence, people with HIGM have an increased susceptibility to infections.<sup id="cite_ref-nat_9-0" class="reference"><a href="#cite_note-nat-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-gen_7-1" class="reference"><a href="#cite_note-gen-7"><span class="cite-bracket">[</span>7<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> <div class="mw-heading mw-heading2"><h2 id="Signs_and_symptoms">Signs and symptoms</h2></div> <p>Hyper IgM syndrome can have the following syndromes:<sup id="cite_ref-emed_6-1" class="reference"><a href="#cite_note-emed-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-11" class="reference"><a href="#cite_note-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup> </p> <ul><li><a href="/wiki/Infection" title="Infection">Infection</a>/<a href="/wiki/Pneumocystis_pneumonia" title="Pneumocystis pneumonia"><i>Pneumocystis</i> pneumonia</a> (PCP), which is common in infants with hyper IgM syndrome, is a serious illness.<sup id="cite_ref-nat_9-1" class="reference"><a href="#cite_note-nat-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> PCP is one of the most frequent and severe opportunistic infections in people with weakened immune systems.</li> <li><a href="/wiki/Hepatitis" title="Hepatitis">Hepatitis</a> (Hepatitis C)</li> <li>Chronic <a href="/wiki/Diarrhea" title="Diarrhea">diarrhea</a></li> <li><a href="/wiki/Hypothyroidism" title="Hypothyroidism">Hypothyroidism</a></li> <li><a href="/wiki/Neutropenia" title="Neutropenia">Neutropenia</a></li> <li><a href="/wiki/Arthritis" title="Arthritis">Arthritis</a></li> <li><a href="/wiki/Encephalopathy" title="Encephalopathy">Encephalopathy</a> (degenerative)</li></ul> <div class="mw-heading mw-heading2"><h2 id="Cause">Cause</h2></div> <figure typeof="mw:File/Thumb"><a href="/wiki/File:Class_switch_recombination.png" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/b/b3/Class_switch_recombination.png/150px-Class_switch_recombination.png" decoding="async" width="150" height="150" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/b/b3/Class_switch_recombination.png/225px-Class_switch_recombination.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/b/b3/Class_switch_recombination.png/300px-Class_switch_recombination.png 2x" data-file-width="750" data-file-height="750" /></a><figcaption>Class switch recombination</figcaption></figure> <p>Different genetic defects cause HIgM syndrome, the vast majority are inherited as an <a href="/wiki/X-linked" class="mw-redirect" title="X-linked">X-linked</a> recessive genetic trait and most sufferers are male.<sup id="cite_ref-gen_7-2" class="reference"><a href="#cite_note-gen-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-om1_1-1" class="reference"><a href="#cite_note-om1-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-om2_2-1" class="reference"><a href="#cite_note-om2-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> <sup id="cite_ref-om3_3-1" class="reference"><a href="#cite_note-om3-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-12" class="reference"><a href="#cite_note-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-om5_4-1" class="reference"><a href="#cite_note-om5-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> </p><p>IgM is the form of <a href="/wiki/Antibody" title="Antibody">antibody</a> that all B cells produce initially before they undergo <a href="/wiki/Antibody#Class_switching" title="Antibody">class switching</a>. Healthy <a href="/wiki/B_cells" class="mw-redirect" title="B cells">B cells</a> efficiently switch to other types of antibodies as needed to attack invading bacteria, viruses, and other pathogens. In people with hyper IgM syndromes, the B cells keep making IgM antibodies because can not switch to a different antibody. This results in an overproduction of IgM antibodies and an underproduction of <a href="/wiki/IgA" class="mw-redirect" title="IgA">IgA</a>, <a href="/wiki/Immunoglobulin_G" title="Immunoglobulin G">IgG</a>, and <a href="/wiki/IgE" class="mw-redirect" title="IgE">IgE</a>.<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><sup id="cite_ref-gen_7-3" class="reference"><a href="#cite_note-gen-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Pathophysiology">Pathophysiology</h2></div> <p>CD40 is a <a href="/wiki/Co-stimulation" title="Co-stimulation">costimulatory</a> receptor on B cells that, when bound to CD40 ligand (<a href="/wiki/CD40L" class="mw-redirect" title="CD40L">CD40L</a>), sends a signal to the <a href="/wiki/B-cell_receptor" title="B-cell receptor">B-cell receptor</a>.<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> When there is a defect in CD40, this leads to defective T-cell interaction with B cells. Consequently, <a href="/wiki/Humoral_immune_response" class="mw-redirect" title="Humoral immune response">humoral immune response</a> is affected. Patients are more susceptible to infection.<sup id="cite_ref-emed_6-2" class="reference"><a href="#cite_note-emed-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Diagnosis">Diagnosis</h2></div> <p>The diagnosis of hyper IgM syndrome can be done via the following methods and tests:<sup id="cite_ref-emed_6-3" class="reference"><a href="#cite_note-emed-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> </p> <ul><li><a href="/wiki/MRI" class="mw-redirect" title="MRI">MRI</a></li> <li>Chest <a href="/wiki/Radiography" title="Radiography">radiography</a></li> <li><a href="/wiki/Pulmonary_function_test" class="mw-redirect" title="Pulmonary function test">Pulmonary function test</a></li> <li><a href="/wiki/Lymph_node" title="Lymph node">Lymph node</a> test</li> <li>Laboratory test (to measure CD40)</li></ul> <div class="mw-heading mw-heading2"><h2 id="Treatment">Treatment</h2></div> <p>In terms of treatment for hyper IgM syndrome, there is the use of <a href="/wiki/Allogeneic" class="mw-redirect" title="Allogeneic">allogeneic</a> <a href="/wiki/Hematopoietic_cell" class="mw-redirect" title="Hematopoietic cell">hematopoietic cell</a> transplantation. Additionally, anti-microbial therapy, use of <a href="/wiki/Granulocyte_colony-stimulating_factor" title="Granulocyte colony-stimulating factor">granulocyte colony-stimulating factor</a>, <a href="/wiki/Immunosuppressants" class="mw-redirect" title="Immunosuppressants">immunosuppressants</a>, as well as other treatments, may be needed.<sup id="cite_ref-gen_7-4" class="reference"><a href="#cite_note-gen-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="References">References</h2></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-om1-1"><span class="mw-cite-backlink">^ <a href="#cite_ref-om1_1-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-om1_1-1"><sup><i><b>b</b></i></sup></a></span> <span class="reference-text"><style data-mw-deduplicate="TemplateStyles:r1238218222">.mw-parser-output cite.citation{font-style:inherit;word-wrap:break-word}.mw-parser-output .citation q{quotes:"\"""\"""'""'"}.mw-parser-output .citation:target{background-color:rgba(0,127,255,0.133)}.mw-parser-output .id-lock-free.id-lock-free a{background:url("//upload.wikimedia.org/wikipedia/commons/6/65/Lock-green.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-limited.id-lock-limited a,.mw-parser-output .id-lock-registration.id-lock-registration a{background:url("//upload.wikimedia.org/wikipedia/commons/d/d6/Lock-gray-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-subscription.id-lock-subscription a{background:url("//upload.wikimedia.org/wikipedia/commons/a/aa/Lock-red-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .cs1-ws-icon a{background:url("//upload.wikimedia.org/wikipedia/commons/4/4c/Wikisource-logo.svg")right 0.1em center/12px no-repeat}body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-free a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-limited a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-registration a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-subscription a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .cs1-ws-icon a{background-size:contain;padding:0 1em 0 0}.mw-parser-output .cs1-code{color:inherit;background:inherit;border:none;padding:inherit}.mw-parser-output .cs1-hidden-error{display:none;color:var(--color-error,#d33)}.mw-parser-output .cs1-visible-error{color:var(--color-error,#d33)}.mw-parser-output .cs1-maint{display:none;color:#085;margin-left:0.3em}.mw-parser-output .cs1-kern-left{padding-left:0.2em}.mw-parser-output .cs1-kern-right{padding-right:0.2em}.mw-parser-output .citation .mw-selflink{font-weight:inherit}@media screen{.mw-parser-output .cs1-format{font-size:95%}html.skin-theme-clientpref-night .mw-parser-output .cs1-maint{color:#18911f}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .cs1-maint{color:#18911f}}</style><cite class="citation web cs1"><a rel="nofollow" class="external text" href="http://www.omim.org/entry/308230">"OMIM Entry - # 308230 - IMMUNODEFICIENCY WITH HYPER-IgM, TYPE 1; HIGM1"</a>. <i>www.omim.org</i><span class="reference-accessdate">. Retrieved <span class="nowrap">16 November</span> 2016</span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=www.omim.org&rft.atitle=OMIM+Entry+-+%23+308230+-+IMMUNODEFICIENCY+WITH+HYPER-IgM%2C+TYPE+1%3B+HIGM1&rft_id=http%3A%2F%2Fwww.omim.org%2Fentry%2F308230&rfr_id=info%3Asid%2Fen.wikipedia.org%3AHyper-IgM+syndrome+type+3" class="Z3988"></span></span> </li> <li id="cite_note-om2-2"><span class="mw-cite-backlink">^ <a href="#cite_ref-om2_2-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-om2_2-1"><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 class="citation web cs1"><a rel="nofollow" class="external text" href="http://omim.org/entry/605258">"OMIM Entry - # 605258 - IMMUNODEFICIENCY WITH HYPER-IgM, TYPE 2; HIGM2"</a>. <i>omim.org</i><span class="reference-accessdate">. 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Rev</i>. <b>203</b>: 48–66. <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.0105-2896.2005.00229.x">10.1111/j.0105-2896.2005.00229.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/15661021">15661021</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Immunol.+Rev.&rft.atitle=Hyper+immunoglobulin+M+syndrome+due+to+CD40+deficiency%3A+clinical%2C+molecular%2C+and+immunological+features&rft.volume=203&rft.pages=48-66&rft.date=2005&rft_id=info%3Adoi%2F10.1111%2Fj.0105-2896.2005.00229.x&rft_id=info%3Apmid%2F15661021&rft.aulast=Lougaris&rft.aufirst=V&rft.au=Badolato%2C+R&rft.au=Ferrari%2C+S&rft.au=Plebani%2C+A&rfr_id=info%3Asid%2Fen.wikipedia.org%3AHyper-IgM+syndrome+type+3" class="Z3988"></span>subscription needed</span> </li> <li id="cite_note-13"><span class="mw-cite-backlink"><b><a href="#cite_ref-13">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFReference" class="citation web cs1">Reference, Genetics Home. <a rel="nofollow" class="external text" href="https://ghr.nlm.nih.gov/condition/x-linked-hyper-igm-syndrome#">"X-linked hyper IgM syndrome"</a>. <i>Genetics Home Reference</i><span class="reference-accessdate">. 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Related Health Problems">ICD</a>-<a href="/wiki/ICD-10" title="ICD-10">10</a></b>: D80.5</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/608106">608106</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/Orphanet" title="Orphanet">Orphanet</a></b>: <a rel="nofollow" class="external text" href="https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=101092">101092</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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title="Template talk:Lymphoid and complement immunodeficiency"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Lymphoid_and_complement_immunodeficiency" title="Special:EditPage/Template:Lymphoid and complement immunodeficiency"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Lymphoid_and_complement_disorders_causing_immunodeficiency" style="font-size:114%;margin:0 4em"><a href="/wiki/Lymphatic_system" title="Lymphatic system">Lymphoid</a> and <a href="/wiki/Complement_system" title="Complement system">complement</a> disorders causing <a href="/wiki/Immunodeficiency" title="Immunodeficiency">immunodeficiency</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Primary_immunodeficiency" title="Primary immunodeficiency">Primary</a></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%"><a href="/wiki/Antibody" title="Antibody">Antibody</a>/<a href="/wiki/Humoral_immune_deficiency" title="Humoral immune deficiency">humoral</a><br />(<a href="/wiki/B_cell" title="B cell">B</a>)</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/Hypogammaglobulinemia" title="Hypogammaglobulinemia">Hypogammaglobulinemia</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><a href="/wiki/X-linked_agammaglobulinemia" title="X-linked agammaglobulinemia">X-linked agammaglobulinemia</a></li> <li><a href="/wiki/Transient_hypogammaglobulinemia_of_infancy" title="Transient hypogammaglobulinemia of infancy">Transient hypogammaglobulinemia of infancy</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Dysgammaglobulinemia" title="Dysgammaglobulinemia">Dysgammaglobulinemia</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/Selective_immunoglobulin_A_deficiency" title="Selective immunoglobulin A deficiency">IgA deficiency</a></li> <li><a href="/wiki/IgG_deficiency" title="IgG deficiency">IgG deficiency</a></li> <li><a href="/wiki/Immunoglobulin_M_deficiency" class="mw-redirect" title="Immunoglobulin M deficiency">IgM deficiency</a></li> <li><a href="/wiki/Hyper_IgM_syndrome" title="Hyper IgM syndrome">Hyper IgM syndrome</a> (<a href="/wiki/Hyper-IgM_syndrome_type_1" title="Hyper-IgM syndrome type 1">1</a></li> <li><a href="/wiki/Hyper-IgM_syndrome_type_2" title="Hyper-IgM syndrome type 2">2</a></li> <li><a class="mw-selflink selflink">3</a></li> <li><a href="/wiki/Hyper-IgM_syndrome_type_4" title="Hyper-IgM syndrome type 4">4</a></li> <li><a href="/wiki/Hyper-IgM_syndrome_type_5" title="Hyper-IgM syndrome type 5">5</a>)</li> <li><a href="/wiki/Wiskott%E2%80%93Aldrich_syndrome" title="Wiskott–Aldrich syndrome">Wiskott–Aldrich syndrome</a></li> <li><a href="/wiki/Hyperimmunoglobulin_E_syndrome" title="Hyperimmunoglobulin E syndrome">Hyper-IgE syndrome</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Other</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/Common_variable_immunodeficiency" title="Common variable immunodeficiency">Common variable immunodeficiency</a></li> <li><a href="/wiki/ICF_syndrome" class="mw-redirect" title="ICF syndrome">ICF syndrome</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/T_cell_deficiency" title="T cell deficiency">T cell deficiency</a><br />(<a href="/wiki/T_cell" title="T cell">T</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><i><a href="/wiki/Thymic_hypoplasia" title="Thymic hypoplasia">thymic hypoplasia</a>:</i> <i>hypoparathyroid</i> (<a href="/wiki/DiGeorge_syndrome" title="DiGeorge syndrome">Di George's syndrome</a>)</li> <li><i>euparathyroid</i> (<a href="/wiki/Nezelof_syndrome" title="Nezelof syndrome">Nezelof syndrome</a></li> <li><a href="/wiki/Ataxia%E2%80%93telangiectasia" title="Ataxia–telangiectasia">Ataxia–telangiectasia</a>)</li></ul> <p><i>peripheral:</i> <a href="/wiki/Purine_nucleoside_phosphorylase_deficiency" title="Purine nucleoside phosphorylase deficiency">Purine nucleoside phosphorylase deficiency</a> </p> <ul><li><a href="/wiki/Hyper_IgM_syndrome" title="Hyper IgM syndrome">Hyper IgM syndrome</a> (<a href="/wiki/Hyper-IgM_syndrome_type_1" title="Hyper-IgM syndrome type 1">1</a>)</li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Severe_combined_immunodeficiency" title="Severe combined immunodeficiency">Severe</a> <a href="/wiki/Combined_immunodeficiencies" title="Combined immunodeficiencies">combined</a><br />(B+T)</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>x-linked:</i> <a href="/wiki/X-linked_severe_combined_immunodeficiency" title="X-linked severe combined immunodeficiency">X-SCID</a><br /><i>autosomal:</i> <a href="/wiki/Adenosine_deaminase_deficiency" title="Adenosine deaminase deficiency">Adenosine deaminase deficiency</a></li> <li><a href="/wiki/Omenn_syndrome" title="Omenn syndrome">Omenn syndrome</a></li> <li><a href="/wiki/ZAP70_deficiency" title="ZAP70 deficiency">ZAP70 deficiency</a></li> <li><a href="/wiki/Bare_lymphocyte_syndrome" title="Bare lymphocyte syndrome">Bare lymphocyte syndrome</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Immunodeficiency#acquired" title="Immunodeficiency">Acquired</a></th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/HIV/AIDS" title="HIV/AIDS">HIV/AIDS</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Leukopenia" title="Leukopenia">Leukopenia</a>:<br /><a href="/wiki/Lymphocytopenia" title="Lymphocytopenia">Lymphocytopenia</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Idiopathic_CD4%2B_lymphocytopenia" title="Idiopathic CD4+ lymphocytopenia">Idiopathic CD4+ lymphocytopenia</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Complement_deficiency" title="Complement deficiency">Complement<br />deficiency</a></th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><i><a href="/wiki/C1-inhibitor" title="C1-inhibitor">C1-inhibitor</a></i> (<a href="/wiki/Angioedema" title="Angioedema">Angioedema</a>/<a href="/wiki/Hereditary_angioedema" title="Hereditary angioedema">Hereditary angioedema</a>)</li> <li><a href="/wiki/Complement_2_deficiency" title="Complement 2 deficiency">Complement 2 deficiency</a>/<a href="/wiki/Complement_4_deficiency" title="Complement 4 deficiency">Complement 4 deficiency</a></li> <li><a href="/wiki/MBL_deficiency" title="MBL deficiency">MBL deficiency</a></li> <li><a href="/wiki/Properdin_deficiency" title="Properdin deficiency">Properdin deficiency</a></li> <li><a href="/wiki/Complement_3_deficiency" title="Complement 3 deficiency">Complement 3 deficiency</a></li> <li><a href="/wiki/Terminal_complement_pathway_deficiency" title="Terminal complement pathway deficiency">Terminal complement pathway deficiency</a></li> <li><a href="/wiki/Paroxysmal_nocturnal_hemoglobinuria" title="Paroxysmal nocturnal hemoglobinuria">Paroxysmal nocturnal hemoglobinuria</a></li> <li><a href="/wiki/Complement_receptor_deficiency" class="mw-redirect" title="Complement receptor deficiency">Complement receptor deficiency</a></li></ul> </div></td></tr></tbody></table></div>    </div><noscript><img src="https://login.wikimedia.org/wiki/Special:CentralAutoLogin/start?type=1x1" alt="" width="1" height="1" style="border: none; position: absolute;"></noscript> <div class="printfooter" data-nosnippet="">Retrieved from "<a dir="ltr" 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