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The severity of <jats:styled-content style=\"fixed-case\">MCA<\/jats:styled-content> depends on a number of different factors, including genetic predisposition, the number and releasability of mast cells involved in the reaction, the type of allergen, presence of specific <jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">E<\/jats:styled-content>, and presence of certain comorbidities. In severe reactions, <jats:styled-content style=\"fixed-case\">MCA<\/jats:styled-content> can be documented by a substantial increase in the serum tryptase level above baseline. When symptoms are recurrent, are accompanied by an increase in mast cell\u2013derived mediators in biological fluids, and are responsive to treatment with mast cell\u2013stabilizing or mediator\u2010targeting drugs, the diagnosis of mast cell activation syndrome (<jats:styled-content style=\"fixed-case\">MCAS<\/jats:styled-content>) is appropriate. Based on the underlying condition, these patients can further be classified into i) primary <jats:styled-content style=\"fixed-case\">MCAS<\/jats:styled-content> where <jats:italic><jats:styled-content style=\"fixed-case\">KIT<\/jats:styled-content><\/jats:italic>\u2010mutated, clonal mast cells are detected, ii) secondary <jats:styled-content style=\"fixed-case\">MCAS<\/jats:styled-content> where an underlying inflammatory disease, often in the form of an <jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">E<\/jats:styled-content>\u2010dependent allergy, but no <jats:italic><jats:styled-content style=\"fixed-case\">KIT<\/jats:styled-content><\/jats:italic>\u2010mutated mast cells, is found, and iii) idiopathic <jats:styled-content style=\"fixed-case\">MCAS<\/jats:styled-content>, where neither an allergy or other underlying disease, nor <jats:italic><jats:styled-content style=\"fixed-case\">KIT<\/jats:styled-content><\/jats:italic>\u2010mutated mast cells are detectable. It is important to note that in many patients with <jats:styled-content style=\"fixed-case\">MCAS<\/jats:styled-content>, several different factors act together to lead to severe or even life\u2010threatening anaphylaxis. Detailed knowledge about the pathogenesis and complexity of <jats:styled-content style=\"fixed-case\">MCAS<\/jats:styled-content>, and thus establishing the exact final diagnosis, may greatly help in the management and therapy of these patients.<\/jats:p>","DOI":"10.1111\/all.12126","type":"journal-article","created":{"date-parts":[[2013,2,15]],"date-time":"2013-02-15T10:30:01Z","timestamp":1360924201000},"page":"417-424","source":"Crossref","is-referenced-by-count":98,"title":["Mast cell activation syndromes: definition and classification"],"prefix":"10.1111","volume":"68","author":[{"given":"P.","family":"Valent","sequence":"first","affiliation":[{"name":"Department of Internal Medicine I Division of Hematology &amp; Hemostaseology Medical University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,2,15]]},"reference":[{"key":"e_1_2_11_2_1","first-page":"3","article-title":"Mast cells and 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