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Systemic mastocytosis (<jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>) is one such condition. Although many <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> patients are suffering from mild or even no mediator\u2010related symptoms, others have recurrent episodes of severe anaphylaxis, with clear signs of a mast cell activation syndrome (<jats:styled-content style=\"fixed-case\">MCAS<\/jats:styled-content>) despite prophylactic therapy with anti\u2010mediator\u2010type drugs. In several of these patients, an IgE\u2010dependent allergy is diagnosed. The severity and frequency of <jats:styled-content style=\"fixed-case\">MCAS<\/jats:styled-content> reactions neither correlate with the burden of neoplastic mast cells nor with the levels of specific IgE or the basal tryptase level. However, there is a relationship between severe anaphylaxis in <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> and the type of allergen. Notably, many of these patients suffer from hymenoptera venom allergy. Currently recommended therapies include the prophylactic use of anti\u2010mediator\u2010type drugs, long\u2010term immunotherapy for hymenoptera venom allergic patients, and epinephrine\u2010self\u2010injector treatment for emergency situations. In patients who present with an excess burden of mast cells, such as smouldering <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>, cytoreductive therapy with cladribine (2CdA) may reduce the frequency of severe events. For the future, additional treatment options, such as IgE\u2010depletion or the use of tyrosine kinase inhibitors blocking IgE\u2010dependent mediator secretion as well as <jats:styled-content style=\"fixed-case\">KIT<\/jats:styled-content> activation, may be useful alternatives.<\/jats:p>","DOI":"10.1111\/cea.12318","type":"journal-article","created":{"date-parts":[[2014,4,5]],"date-time":"2014-04-05T07:33:03Z","timestamp":1396683183000},"page":"914-920","source":"Crossref","is-referenced-by-count":42,"title":["Risk factors and management of severe life\u2010threatening anaphylaxis in patients with clonal mast cell disorders"],"prefix":"10.1111","volume":"44","author":[{"given":"P.","family":"Valent","sequence":"first","affiliation":[{"name":"Department of Internal Medicine I Division of Hematology &amp; Hemostaseology Medical University of Vienna Austria and Ludwig Boltzmann Cluster Oncology Medical University of Vienna Vienna Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2014,6,23]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12468882"},{"key":"e_1_2_11_3_1","first-page":"385","article-title":"Biology, classification and treatment of human mastocytosis","volume":"108","author":"Valent P","year":"1996","journal-title":"Wien Klin Wochenschr"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2003.04575.x"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.med.55.091902.103822"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0145-2126(01)00021-2"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1586\/ehm.10.42"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.92.23.10560"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0396-312"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1159\/000048179"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.02783.x"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2009.281"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V91.8.2731.2731_2731_2736"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-065X.2001.790108.x"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1053\/hupa.2001.24319"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.pas.0000138181.89743.7b"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI118337"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198706253162603"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1159\/000059404"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0145-2126(01)00038-8"},{"key":"e_1_2_11_21_1","first-page":"291","volume-title":"World Health Organization (WHO) Classification of tumours. 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