{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T01:17:51Z","timestamp":1790471871033,"version":"4.1.0"},"reference-count":37,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2014,8,21]],"date-time":"2014-08-21T00:00:00Z","timestamp":1408579200000},"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":[[2014,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The mechanisms by which mast cells in patients with unexplained anaphylaxis (<jats:styled-content style=\"fixed-case\">UEA<\/jats:styled-content>) are triggered remain elusive. Onset of episodes is unpredictable and often recurrent. The substantial overlap between the clinical manifestations of<jats:styled-content style=\"fixed-case\">UEA<\/jats:styled-content>and clonal mast cell disorders (<jats:styled-content style=\"fixed-case\">CMD<\/jats:styled-content>) suggests an association between these rare disorders. The two forms of<jats:styled-content style=\"fixed-case\">CMD<\/jats:styled-content>characterized to date are systemic mastocytosis (<jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>) and monoclonal mast cell activation syndrome (<jats:styled-content style=\"fixed-case\">MMAS<\/jats:styled-content>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To examine the hypothesis that the pathogenesis of<jats:styled-content style=\"fixed-case\">UEA<\/jats:styled-content>reflects the presence of aberrant subpopulations of mast cells.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Thirty (14 men, 16 women) patients (\u2265\u00a018\u00a0years) suffering from UEA and with no signs of cutaneous mastocytosis were recruited. Patients underwent an initial complete allergy work\u2010up to confirm the diagnosis of UEA. Level of baseline serum tryptase (sBT) and total IgE were determined. In addition, a bone marrow examination was performed on all 30 patients to investigate possible underlying CMD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fourteen (47%) of our cases (nine men, five women) were diagnosed with CMD: 10 with SM and four with MMAS. Four of the 10 patients with SM had mast cell aggregates in their bone marrow. All patients with SM exhibited a sBT level &gt;\u00a011.4\u00a0ng\/mL, whereas this level was elevated in only two of those with MMAS and four with UAE but not diagnosed with CMD. Total IgE levels were lower in the group of patients with CMD (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.03).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion and Clinical Relevance<\/jats:title><jats:p>The pathogenic mechanism underlying UEA could be explained by the presence of immunophenotypically aberrant mast cells with clonal markers in 47% of our subjects, indicating that clonal mast cell disorders are present in a substantial subset of these patients. Thus, the presence of CMD should be considered in patients with UEA if they have an elevated level of sBT (\u2265\u00a011.4\u00a0ng\/mL) and cardiovascular symptoms such as syncope.<\/jats:p><\/jats:sec>","DOI":"10.1111\/cea.12369","type":"journal-article","created":{"date-parts":[[2014,7,5]],"date-time":"2014-07-05T08:15:23Z","timestamp":1404548123000},"page":"1179-1187","source":"Crossref","is-referenced-by-count":80,"title":["The presence of mast cell clonality in patients with unexplained anaphylaxis"],"prefix":"10.1111","volume":"44","author":[{"given":"T.","family":"G\u00fclen","sequence":"first","affiliation":[{"name":"Department of Medicine Solna Clinical Immunology and Allergy Research Unit Karolinska Institutet Stockholm Sweden"},{"name":"Department of Respiratory Medicine and Allergy Karolinska University Hospital Huddinge Stockholm Sweden"},{"name":"Mastocytosis Centre Karolinska Karolinska University Hospital and 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 and Karolinska Institutet Stockholm Sweden"},{"name":"Department of Hematology Karolinska University Hospital Huddinge Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Sander","sequence":"additional","affiliation":[{"name":"Mastocytosis Centre Karolinska Karolinska University Hospital and Karolinska Institutet Stockholm Sweden"},{"name":"Department of Pathology 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 and 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 Solna Clinical Immunology and Allergy Research Unit Karolinska Institutet Stockholm Sweden"},{"name":"Mastocytosis Centre Karolinska Karolinska University Hospital and 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":[[2014,8,21]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2010.06.022"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2222.1978.tb03227.x"},{"key":"e_1_2_7_4_1","first-page":"53","article-title":"Anaphylaxis: clinical aspects, etiology and course in 118 patients","volume":"128","author":"Rohrer CL","year":"1998","journal-title":"Schweiz Med Wochenschr"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1081-1206(10)61367-1"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.2500\/aap.2012.33.3557"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12462239"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1081-1206(10)62547-1"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-06-028100"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2008.11.018"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2010.02.019"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1159\/000096442"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2362.2007.01807.x"},{"key":"e_1_2_7_14_1","first-page":"54","volume-title":"World Health Organization (WHO) classification of tumours, pathology & genetics. 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