{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T20:46:32Z","timestamp":1790628392157,"version":"4.1.0"},"reference-count":37,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2013,12,20]],"date-time":"2013-12-20T00:00:00Z","timestamp":1387497600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clin Experimental Allergy"],"published-print":{"date-parts":[[2014,1]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Systemic mastocytosis (<jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>) is a clonal mast cells disorder characterized by the proliferation, accumulation and activation of mast cells in extracutaneous tissues. The clinical picture is heterogeneous and may range from asymptomatic to potentially fatal anaphylactic reactions due to excessive mast cell mediator release.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>The aim of this study was to investigate the prevalence and trigger factors of anaphylactic reactions among adult <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> patients. We also explored the clinical spectrum of mast cell mediator\u2010related symptoms in patients with <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This descriptive study was performed among 84 consecutive adult (\u2265\u00a018\u00a0years) patients those were diagnosed with <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> according to <jats:styled-content style=\"fixed-case\">WHO<\/jats:styled-content> criteria. Sixty\u2010six of the patients also underwent a comprehensive allergy work\u2010up.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sixty of 84 patients with <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> (71%) had bone marrow mast cell aggregates and fulfilled the major criteria for <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> and 76 patients (91%) had indolent disease. Simultaneous occurrence of cutaneous mastocytosis was observed in 59 patients (70%). Thirty\u2010six patients (43%) had had at least one episode of an anaphylactic reaction. The clinical courses of the reactions were usually severe and patients often presented with syncope attacks (72%). Most patients reacted after hymenoptera venom stings (19\/36; 53%). In 39% (14\/36), a clear aetiology could not be determined. While males and females were equally frequent among the patients with <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>, anaphylaxis patients showed a male predominance (61%). Anaphylactic reactions occurred more frequently in patients without cutaneous engagement. The rate of allergy sensitization was significantly higher in <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> patients with anaphylaxis as compared with non\u2010anaphylaxis <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> patients, 70% vs. 23%, respectively (<jats:italic>P\u00a0<\/jats:italic>=\u00a00.0002).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions and Clinical Relevance<\/jats:title><jats:p>Anaphylaxis is more prevalent in patients with <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>, predominantly in patients with atopic <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>. Hymenoptera venom\u2010induced and idiopathic anaphylaxis were the most frequent elicitors. Our findings implicate that all mastocytosis patients with anaphylaxis should undergo detailed allergological assessment before considering treatment and preventive measures.<\/jats:p><\/jats:sec>","DOI":"10.1111\/cea.12225","type":"journal-article","created":{"date-parts":[[2013,10,25]],"date-time":"2013-10-25T17:14:35Z","timestamp":1382721275000},"page":"121-129","source":"Crossref","is-referenced-by-count":143,"title":["High prevalence of anaphylaxis in patients with systemic mastocytosis \u2013 a single\u2010centre experience"],"prefix":"10.1111","volume":"44","author":[{"given":"T.","family":"G\u00fclen","sequence":"first","affiliation":[{"name":"Department of Respiratory Medicine and Allergy Karolinska University Hospital Huddinge Stockholm Sweden"},{"name":"Department of Medicine Clinical Immunology and Allergy Research Unit Karolinska University Hospital Karolinska Institutet Solna Stockholm Sweden"},{"name":"Mastocytosis Centre Karolinska Karolinska University Hospital Karolinska Institutet Stockholm Sweden"},{"name":"Centre for Allergy Research (CfA) Karolinska Institutet Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"H\u00e4gglund","sequence":"additional","affiliation":[{"name":"Mastocytosis Centre Karolinska Karolinska University Hospital Karolinska Institutet Stockholm Sweden"},{"name":"Department of Haematology Karolinska University Hospital Huddinge Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Dahl\u00e9n","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Allergy Karolinska University Hospital Huddinge Stockholm Sweden"},{"name":"Mastocytosis Centre Karolinska Karolinska University Hospital Karolinska Institutet Stockholm Sweden"},{"name":"Centre for Allergy Research (CfA) Karolinska Institutet Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Nilsson","sequence":"additional","affiliation":[{"name":"Department of Medicine Clinical Immunology and Allergy Research Unit Karolinska University Hospital Karolinska Institutet Solna Stockholm Sweden"},{"name":"Mastocytosis Centre Karolinska Karolinska University Hospital Karolinska Institutet Stockholm Sweden"},{"name":"Centre for Allergy Research (CfA) Karolinska Institutet Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,12,20]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2005.12.1303"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0091-6749(99)70392-1"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2222.2004.01882.x"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2003.11.033"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2008.09.043"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1081-1206(10)61086-1"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0145-2126(01)00038-8"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2362.2007.01807.x"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.med.55.091902.103822"},{"key":"e_1_2_7_11_1","first-page":"54","volume-title":"World Health Organization (WHO) Classification of Tumours, Pathology & Genetics. 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