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window.loswp.showSignupCaptcha = false window.loswp.willEdgeCache = false; window.loswp.work = {"work":{"id":118082294,"created_at":"2024-04-25T21:17:01.438-07:00","from_world_paper_id":253462613,"updated_at":"2025-01-11T15:46:17.818-08:00","_data":{"publisher":"Elsevier BV","ai_abstract":"This case series evaluates the effectiveness of Omalizumab in treating Non-Clonal Mast Cell Activation Syndrome (nc-MCAS). Findings indicate that Omalizumab is both safe and effective, showing improvements in symptoms and quality of life for patients with nc-MCAS after treatment initiation. Notably, anaphylaxis prevention occurs from the first injection, and the clinical benefits may persist even after treatment cessation.","publication_date":"2020,,","publication_name":"European Journal of Internal Medicine"},"document_type":"paper","pre_hit_view_count_baseline":null,"quality":"high","language":"en","title":"Timing of response and long term efficacy of Omalizumab in non-clonal Mast Cell Activation Syndrome: A case series","broadcastable":false,"draft":null,"has_indexable_attachment":true,"indexable":true}}["work"]; window.loswp.workCoauthors = [42988908]; window.loswp.locale = "en"; window.loswp.countryCode = "SG"; window.loswp.cwvAbTestBucket = ""; window.loswp.designVariant = "ds_vanilla"; window.loswp.fullPageMobileSutdModalVariant = "control"; window.loswp.useOptimizedScribd4genScript = false; window.loginModal = {}; window.loginModal.appleClientId = 'edu.academia.applesignon'; window.userInChina = "false";</script><script defer="" src="https://accounts.google.com/gsi/client"></script><div class="ds-loswp-container"><div class="ds-work-card--grid-container"><div class="ds-work-card--container js-loswp-work-card"><div class="ds-work-card--cover"><div class="ds-work-cover--wrapper"><div class="ds-work-cover--container"><button class="ds-work-cover--clickable js-swp-download-button" data-signup-modal="{"location":"swp-splash-paper-cover","attachmentId":113790912,"attachmentType":"pdf"}"><img alt="First page of “Timing of response and long term efficacy of Omalizumab in non-clonal Mast Cell Activation Syndrome: A case series”" class="ds-work-cover--cover-thumbnail" src="https://0.academia-photos.com/attachment_thumbnails/113790912/mini_magick20240802-1-5zi207.png?1722613611" /><img alt="PDF Icon" class="ds-work-cover--file-icon" src="//a.academia-assets.com/images/single_work_splash/adobe_icon.svg" /><div class="ds-work-cover--hover-container"><span class="material-symbols-outlined" style="font-size: 20px" translate="no">download</span><p>Download Free PDF</p></div><div class="ds-work-cover--ribbon-container">Download Free PDF</div><div class="ds-work-cover--ribbon-triangle"></div></button></div></div></div><div class="ds-work-card--work-information"><h1 class="ds-work-card--work-title">Timing of response and long term efficacy of Omalizumab in non-clonal Mast Cell Activation Syndrome: A case series</h1><div class="ds-work-card--work-authors ds-work-card--detail"><a class="ds-work-card--author js-wsj-grid-card-author ds2-5-body-md ds2-5-body-link" data-author-id="42988908" href="https://azospvr.academia.edu/MicheleSchiappoli"><img alt="Profile image of Michele Schiappoli" class="ds-work-card--author-avatar" src="https://0.academia-photos.com/42988908/175371266/165412130/s65_michele.schiappoli.jpeg" />Michele Schiappoli</a></div><div class="ds-work-card--detail"><p class="ds-work-card--detail ds2-5-body-sm">2020, European Journal of Internal Medicine</p><div class="ds-work-card--work-metadata"><div class="ds-work-card--work-metadata__stat"><span class="material-symbols-outlined" style="font-size: 20px" translate="no">visibility</span><p class="ds2-5-body-sm" id="work-metadata-view-count">…</p></div><div class="ds-work-card--work-metadata__stat"><span class="material-symbols-outlined" style="font-size: 20px" translate="no">description</span><p class="ds2-5-body-sm">3 pages</p></div><div class="ds-work-card--work-metadata__stat"><span class="material-symbols-outlined" style="font-size: 20px" translate="no">link</span><p class="ds2-5-body-sm">1 file</p></div></div><script>(async () => { const workId = 118082294; 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Findings indicate that Omalizumab is both safe and effective, showing improvements in symptoms and quality of life for patients with nc-MCAS after treatment initiation. Notably, anaphylaxis prevention occurs from the first injection, and the clinical benefits may persist even after treatment cessation.</p><div class="ds-work-card--button-container"><button class="ds2-5-button js-swp-download-button" data-signup-modal="{"location":"continue-reading-button--work-card","attachmentId":113790912,"attachmentType":"pdf","workUrl":"https://www.academia.edu/118082294/Timing_of_response_and_long_term_efficacy_of_Omalizumab_in_non_clonal_Mast_Cell_Activation_Syndrome_A_case_series"}">See full PDF</button><button class="ds2-5-button ds2-5-button--secondary js-swp-download-button" data-signup-modal="{"location":"download-pdf-button--work-card","attachmentId":113790912,"attachmentType":"pdf","workUrl":"https://www.academia.edu/118082294/Timing_of_response_and_long_term_efficacy_of_Omalizumab_in_non_clonal_Mast_Cell_Activation_Syndrome_A_case_series"}"><span class="material-symbols-outlined" style="font-size: 20px" translate="no">download</span>Download PDF</button></div><div class="ds-signup-banner-trigger-container"><div class="ds-signup-banner-trigger ds-signup-banner-trigger-control"></div></div><div class="ds-signup-banner ds-signup-banner-control"><div id="ds-signup-banner-close-button"><button class="ds2-5-button ds2-5-button--secondary ds2-5-button--inverse"><span class="material-symbols-outlined" style="font-size: 20px" translate="no">close</span></button></div><div class="ds-signup-banner-ctas"><img src="//a.academia-assets.com/images/academia-logo-capital-white.svg" /><h4 class="ds2-5-heading-serif-sm">Sign up for access to the world's latest research</h4><button class="ds2-5-button ds2-5-button--inverse ds2-5-button--full-width js-swp-download-button" data-signup-modal="{"location":"signup-banner"}">Sign up for free<span class="material-symbols-outlined" style="font-size: 20px" translate="no">arrow_forward</span></button></div><div class="ds-signup-banner-divider"></div><div class="ds-signup-banner-reasons"><div class="ds-signup-banner-reasons-item"><span class="material-symbols-outlined" style="font-size: 24px" translate="no">check</span><span>Get notified about relevant papers</span></div><div class="ds-signup-banner-reasons-item"><span class="material-symbols-outlined" style="font-size: 24px" translate="no">check</span><span>Save papers to use in your research</span></div><div class="ds-signup-banner-reasons-item"><span class="material-symbols-outlined" style="font-size: 24px" translate="no">check</span><span>Join the discussion with peers</span></div><div class="ds-signup-banner-reasons-item"><span class="material-symbols-outlined" style="font-size: 24px" translate="no">check</span><span>Track your impact</span></div></div></div><script>(() => { // Set up signup banner show/hide behavior: // 1. 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MCA-related symptoms range from mild to severe to life-threatening. The severity of MCA-related symptoms depends on a number of factors, including genetic predisposition, the number and releasability of MCs, organs affected, and the type and consequences of comorbid conditions. In severe systemic reactions, MCA is demonstrable by a substantial increase of basal serum tryptase levels above the individual’s baseline. When, in addition, the symptoms are recurrent, involve more than one organ system, and are responsive to therapy with MC-stabilizing or mediator-targeting drugs, the consensus criteria for the diagnosis of MCA syndrome (MCAS) are met. Based on the etiology of MCA, patients can further be classified as having i) primary MCAS...</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{"location":"wsj-grid-card-download-pdf-modal","work_title":"Diagnosis, Classification and Management of Mast Cell Activation Syndromes (MCAS) in the Era of Personalized Medicine","attachmentId":75937304,"attachmentType":"pdf","work_url":"https://www.academia.edu/63539780/Diagnosis_Classification_and_Management_of_Mast_Cell_Activation_Syndromes_MCAS_in_the_Era_of_Personalized_Medicine","alternativeTracking":true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/63539780/Diagnosis_Classification_and_Management_of_Mast_Cell_Activation_Syndromes_MCAS_in_the_Era_of_Personalized_Medicine"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="2" data-entity-id="77395593" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/77395593/Mast_cell_activation_syndrome_High_frequency_of_skin_manifestations_and_anaphylactic_shock">Mast cell activation syndrome: High frequency of skin manifestations and anaphylactic shock</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="35302466" href="https://independent.academia.edu/OlivierHermine">Olivier Hermine</a></div><p class="ds-related-work--metadata ds2-5-body-xs">Allergology international : official journal of the Japanese Society of Allergology, 2018</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{"location":"wsj-grid-card-download-pdf-modal","work_title":"Mast cell activation syndrome: High frequency of skin manifestations and anaphylactic shock","attachmentId":84772703,"attachmentType":"pdf","work_url":"https://www.academia.edu/77395593/Mast_cell_activation_syndrome_High_frequency_of_skin_manifestations_and_anaphylactic_shock","alternativeTracking":true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/77395593/Mast_cell_activation_syndrome_High_frequency_of_skin_manifestations_and_anaphylactic_shock"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="3" data-entity-id="18413564" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/18413564/Definitions_Criteria_and_Global_Classification_of_Mast_Cell_Disorders_with_Special_Reference_to_Mast_Cell_Activation_Syndromes_A_Consensus_Proposal">Definitions, Criteria and Global Classification of Mast Cell Disorders with Special Reference to Mast Cell Activation Syndromes: A Consensus Proposal</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="38243327" href="https://independent.academia.edu/BoguslawNedoszytko">Boguslaw Nedoszytko</a></div><p class="ds-related-work--metadata ds2-5-body-xs">International Archives of Allergy and Immunology, 2012</p><p class="ds-related-work--abstract ds2-5-body-sm">for diagnosing mastocytosis are not met. A working conference was organized in 2010 with the aim to define criteria for diagnosing MCA and related disorders, and to propose a global unifying classification of all MC disorders and pathologic MC reactions. This classification includes three types of 'MCA syndromes' (MCASs), namely primary MCAS, secondary MCAS and idiopathic MCAS. MCA is now defined by robust and generally applicable criteria, including (1) typical clinical symptoms, (2) a substantial transient increase in serum total tryptase level or an increase in other MC-derived mediators, such as histamine or prostaglandin D 2 , or their urinary metabolites, and (3) a response of clinical symptoms to agents that attenuate the production or activities of MC mediators. These criteria should assist in the identification Abstract Activation of tissue mast cells (MCs) and their abnormal growth and accumulation in various organs are typically found in primary MC disorders also referred to as mastocytosis. However, increasing numbers of patients are now being informed that their clinical findings are due to MC activation (MCA) that is neither associated with mastocytosis nor with a defined allergic or inflammatory reaction. In other patients with MCA, MCs appear to be clonal cells, but criteria Valent et al. Int Arch Allergy Immunol 2012;157:215-225 216 and diagnosis of patients with MCAS, and in avoiding misdiagnoses or overinterpretation of clinical symptoms in daily practice. Moreover, the MCAS concept should stimulate research in order to identify and exploit new molecular mechanisms and therapeutic targets.</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{"location":"wsj-grid-card-download-pdf-modal","work_title":"Definitions, Criteria and Global Classification of Mast Cell Disorders with Special Reference to Mast Cell Activation Syndromes: A Consensus Proposal","attachmentId":40051091,"attachmentType":"pdf","work_url":"https://www.academia.edu/18413564/Definitions_Criteria_and_Global_Classification_of_Mast_Cell_Disorders_with_Special_Reference_to_Mast_Cell_Activation_Syndromes_A_Consensus_Proposal","alternativeTracking":true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/18413564/Definitions_Criteria_and_Global_Classification_of_Mast_Cell_Disorders_with_Special_Reference_to_Mast_Cell_Activation_Syndromes_A_Consensus_Proposal"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="4" data-entity-id="100102500" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/100102500/Selecting_the_Right_Criteria_and_Proper_Classification_to_Diagnose_Mast_Cell_Activation_Syndromes_A_Critical_Review">Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="35348000" href="https://independent.academia.edu/Bogus%C5%82awNedoszytko">Bogusław Nedoszytko</a></div><p class="ds-related-work--metadata ds2-5-body-xs">The Journal of Allergy and Clinical Immunology: In Practice, 2021</p><p class="ds-related-work--abstract ds2-5-body-sm">In recent years, knowledge about mechanisms underlying mast cell activation (MCA) and accumulation in various pathologic conditions increased substantially. In addition, criteria and a classification of MCA syndromes (MCASs) have been set forth. MCAS is defined by typical clinical symptoms, a substantial increase in serum tryptase level during an attack over the patient's baseline tryptase, and a response of the symptoms to drugs targeting mast cells, mediator production, and/or</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{"location":"wsj-grid-card-download-pdf-modal","work_title":"Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review","attachmentId":101016053,"attachmentType":"pdf","work_url":"https://www.academia.edu/100102500/Selecting_the_Right_Criteria_and_Proper_Classification_to_Diagnose_Mast_Cell_Activation_Syndromes_A_Critical_Review","alternativeTracking":true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/100102500/Selecting_the_Right_Criteria_and_Proper_Classification_to_Diagnose_Mast_Cell_Activation_Syndromes_A_Critical_Review"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="5" data-entity-id="23142729" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/23142729/Mast_cell_activation_syndrome_A_newly_recognized_disorder_with_systemic_clinical_manifestations">Mast cell activation syndrome: A newly recognized disorder with systemic clinical manifestations</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="44919922" href="https://independent.academia.edu/NortonGreenberger">Norton Greenberger</a><span>, </span><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="44977311" href="https://independent.academia.edu/MatthewHamilton13">Matthew Hamilton</a></div><p class="ds-related-work--metadata ds2-5-body-xs">Journal of Allergy and Clinical Immunology, 2011</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{"location":"wsj-grid-card-download-pdf-modal","work_title":"Mast cell activation syndrome: A newly recognized disorder with systemic clinical manifestations","attachmentId":43630752,"attachmentType":"pdf","work_url":"https://www.academia.edu/23142729/Mast_cell_activation_syndrome_A_newly_recognized_disorder_with_systemic_clinical_manifestations","alternativeTracking":true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/23142729/Mast_cell_activation_syndrome_A_newly_recognized_disorder_with_systemic_clinical_manifestations"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="6" data-entity-id="115119790" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/115119790/Nonclonal_Mast_Cell_Activation_Syndrome">Nonclonal Mast Cell Activation Syndrome</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="44977311" href="https://independent.academia.edu/MatthewHamilton13">Matthew Hamilton</a></div><p class="ds-related-work--metadata ds2-5-body-xs">Immunology and Allergy Clinics of North America, 2018</p><p class="ds-related-work--abstract ds2-5-body-sm">Patients who present with typical features of mast cell activation with laboratory confirmation and without evidence of a clonal mast cell disorder or other medical condition should be initiated on medical treatment to block mast cells and their mediators. If a major response is achieved, a diagnosis of non-clonal mast cell activation syndrome (NC-MCAS) is likely and treatment should be optimized including management of any associated conditions. In this review, the latest evidence with regards to the diagnosis and treatment of NC-MCAS is presented.</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{"location":"wsj-grid-card-download-pdf-modal","work_title":"Nonclonal Mast Cell Activation Syndrome","attachmentId":111621245,"attachmentType":"pdf","work_url":"https://www.academia.edu/115119790/Nonclonal_Mast_Cell_Activation_Syndrome","alternativeTracking":true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/115119790/Nonclonal_Mast_Cell_Activation_Syndrome"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="7" data-entity-id="78090431" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/78090431/Clinical_biological_and_molecular_characteristics_of_clonal_mast_cell_disorders_presenting_with_systemic_mast_cell_activation_symptoms">Clinical, biological, and molecular characteristics of clonal mast cell disorders presenting with systemic mast cell activation symptoms</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="35593572" href="https://independent.academia.edu/EscribanoL">L. Escribano</a></div><p class="ds-related-work--metadata ds2-5-body-xs">Journal of Allergy and Clinical Immunology, 2010</p><p class="ds-related-work--abstract ds2-5-body-sm">Background: Systemic mast cell activation disorders (MCADs) are characterized by severe and systemic mast cell (MC) mediators-related symptoms frequently associated with increased serum baseline tryptase (sBt). Objective: To analyze the clinical, biological, and molecular characteristics of adult patients presenting with systemic MC activation symptoms/anaphylaxis in the absence of skin mastocytosis who showed clonal (c) versus nonclonal (nc) MCs and to provide indication criteria for bone marrow (BM) studies. Methods: Eighty-three patients were studied. Patients showing clonal BM MCs were grouped into indolent systemic mastocytosis without skin lesions (ISMs-; n 5 48) and other c-MCADs (n 5 3)-both with CD25 11 BM MCs and either positive mast/stem cell growth factor receptor gene (KIT) mutation or clonal human androgen receptor assay (HUMARA) tests-and nc-MCAD (CD25-negative BM MCs in the absence of KIT mutation; n 5 32) and compared for their clinical, biological, and molecular characteristics. Results: Most clonal patients (48/51; 94%) met the World Health Organization criteria for systemic mastocytosis and were classified as ISMs-, whereas the other 3 c-MCAD and all nc-MCAD patients did not. In addition, although both patients with ISMsand patients with nc-MCAD presented with idiopathic and allergen-induced anaphylaxis, the former showed a higher frequency of men, cardiovascular symptoms, and insect bite as a trigger, together with greater sBt. Based on a multivariate analysis, a highly efficient model to predict clonality before BM sampling was built that includes male sex (P 5 .01), presyncopal and/or syncopal episodes (P 5 .009) in the absence of urticaria and angioedema (P 5 .003), and sBt >25 mg/L (P 5 .006) as independent predictive factors. Conclusions: Patients with c-MCAD and ISMsdisplay unique clinical and laboratory features different from nc-MCAD patients. A significant percentage of c-MCAD patients can be considered as true ISMsdiagnosed at early phases of the disease.</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{"location":"wsj-grid-card-download-pdf-modal","work_title":"Clinical, biological, and molecular characteristics of clonal mast cell disorders presenting with systemic mast cell activation symptoms","attachmentId":85259546,"attachmentType":"pdf","work_url":"https://www.academia.edu/78090431/Clinical_biological_and_molecular_characteristics_of_clonal_mast_cell_disorders_presenting_with_systemic_mast_cell_activation_symptoms","alternativeTracking":true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/78090431/Clinical_biological_and_molecular_characteristics_of_clonal_mast_cell_disorders_presenting_with_systemic_mast_cell_activation_symptoms"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="8" data-entity-id="94692950" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/94692950/Clinical_Approach_to_Mast_Cell_Activation_Syndromes_A_Practical_Overview">Clinical Approach to Mast Cell Activation Syndromes: A Practical Overview</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="172841658" href="https://independent.academia.edu/MARINAGORDO">MARINA GORDO</a></div><p class="ds-related-work--metadata ds2-5-body-xs">Journal of investigational allergology & clinical immunology, 2021</p><p class="ds-related-work--abstract ds2-5-body-sm">The diagnosis of mast cell activation syndromes (MCAS) is defined by 3 criteria: 1) typical clinical signs and symptoms of acute, recurrent (episodic) and systemic mast cell activation (MCA), 2) increase in tryptase level to plus 20 % + 2 ng/ml within a 1-4 hours after onset of the acute crisis, 3) response of MCA symptoms to antimediator therapy. Classification of MCAS requires applying highly sensitive and specific methodological approaches for assessing clonal bone marrow mast cells (BM MCs) at low frequencies. The Spanish Network on Mastocytosis (REMA) score is successfully used as predictive model for selecting MCAS candidates for BM studies based on a high probability of having an underlying clonal mast cell disorder (c-MCD). In this article, we propose a diagnostic algorithm and focus in the practical evaluation and management of patients with suspected MCAS.</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{"location":"wsj-grid-card-download-pdf-modal","work_title":"Clinical Approach to Mast Cell Activation Syndromes: A Practical Overview","attachmentId":97079668,"attachmentType":"pdf","work_url":"https://www.academia.edu/94692950/Clinical_Approach_to_Mast_Cell_Activation_Syndromes_A_Practical_Overview","alternativeTracking":true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/94692950/Clinical_Approach_to_Mast_Cell_Activation_Syndromes_A_Practical_Overview"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="9" data-entity-id="74447237" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/74447237/AAAAI_Mast_Cell_Disorders_Committee_Work_Group_Report_Mast_Cell_Activation_Syndrome_MCAS_Diagnosis_and_Management">AAAAI Mast Cell Disorders Committee Work Group Report: Mast Cell Activation Syndrome (MCAS) Diagnosis and Management</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="107605558" href="https://mssm.academia.edu/AnneMaitland">Anne Maitland</a></div><p class="ds-related-work--metadata ds2-5-body-xs">Journal of Allergy and Clinical Immunology</p><p class="ds-related-work--abstract ds2-5-body-sm">AAAAI Position Statements,Work Group Reports, and Systematic Reviews are not to be considered to reflect current AAAAI standards or policy after five years from the date of publication. The statement below is not to be construed as dictating an exclusive course of action nor is it intended to replace the medical judgment of healthcare professionals. The unique circumstances of individual patients and environments are to be taken into account in any diagnosis and treatment plan. The statement reflects clinical and scientific advances as of the date of publication and is subject to change.</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{"location":"wsj-grid-card-download-pdf-modal","work_title":"AAAAI Mast Cell Disorders Committee Work Group Report: Mast Cell Activation Syndrome (MCAS) Diagnosis and Management","attachmentId":82597620,"attachmentType":"pdf","work_url":"https://www.academia.edu/74447237/AAAAI_Mast_Cell_Disorders_Committee_Work_Group_Report_Mast_Cell_Activation_Syndrome_MCAS_Diagnosis_and_Management","alternativeTracking":true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/74447237/AAAAI_Mast_Cell_Disorders_Committee_Work_Group_Report_Mast_Cell_Activation_Syndrome_MCAS_Diagnosis_and_Management"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div></div></div><div class="ds-sticky-ctas--wrapper js-loswp-sticky-ctas hidden"><div class="ds-sticky-ctas--grid-container"><div class="ds-sticky-ctas--container"><button class="ds2-5-button js-swp-download-button" data-signup-modal="{"location":"continue-reading-button--sticky-ctas","attachmentId":113790912,"attachmentType":"pdf","workUrl":null}">See full PDF</button><button class="ds2-5-button ds2-5-button--secondary js-swp-download-button" data-signup-modal="{"location":"download-pdf-button--sticky-ctas","attachmentId":113790912,"attachmentType":"pdf","workUrl":null}"><span class="material-symbols-outlined" style="font-size: 20px" translate="no">download</span>Download PDF</button></div></div></div><div class="ds-below-fold--grid-container"><div class="ds-work--container js-loswp-embedded-document"><div class="attachment_preview" data-attachment="Attachment_113790912" style="display: none"><div class="js-scribd-document-container"><div class="scribd--document-loading js-scribd-document-loader" style="display: block;"><img alt="Loading..." src="//a.academia-assets.com/images/loaders/paper-load.gif" /><p>Loading Preview</p></div></div><div style="text-align: center;"><div class="scribd--no-preview-alert js-preview-unavailable"><p>Sorry, preview is currently unavailable. 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