{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T03:52:46Z","timestamp":1792641166676,"version":"4.5.0"},"reference-count":14,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2014,4,25]],"date-time":"2014-04-25T00:00:00Z","timestamp":1398384000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Blueprint Medicines, Cambridge, MA, USA"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2014,8]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Mastocytosis is a heterogeneous group of diseases characterized by abnormal proliferation of mast cells. Systemic mastocytosis (<jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>), in which abnormal mast cells are present in tissues beyond the skin, is divided into seven subcategories with varying degrees of severity and prognosis. Very little is known about the epidemiology of <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> and its subcategories. This retrospective cohort study of 548 adults with <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> diagnosed 1997\u20132010 was constructed using linked Danish national health registries. The most common subtype of mastocytosis was indolent <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> (including urticaria pigmentosa) (<jats:italic>n<\/jats:italic>\u00a0=\u00a0450; 82%), followed by <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> with subtype unknown (<jats:italic>n<\/jats:italic>\u00a0=\u00a061; 11%), <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> with associated clonal haematological non\u2010mast cell lineage disease (<jats:italic>n<\/jats:italic>\u00a0=\u00a024; 4%), aggressive <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> (<jats:italic>n<\/jats:italic>\u00a0=\u00a08; 2%), and mast cell leukaemia (<jats:italic>n<\/jats:italic>\u00a0=\u00a05; 1%). The incidence rate for <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> (all subtypes including urticaria pigmentosa) was 0\u00b789 per 100\u00a0000 per year. Cumulative incidence was 12\u00b746 per 100\u00a0000, and the 14\u2010year limited\u2010duration prevalence as of 1 January, 2011 was 9\u00b759 per 100\u00a0000. This nationwide cohort from Denmark is the first population\u2010based epidemiological study of mastocytosis. In this cohort of patients aged 15\u00a0years and older, <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> was found to be overall relatively rare with notable variation by subtype for patient characteristics, survival and epidemiological measures.<\/jats:p>","DOI":"10.1111\/bjh.12916","type":"journal-article","created":{"date-parts":[[2014,4,25]],"date-time":"2014-04-25T06:52:49Z","timestamp":1398408769000},"page":"521-528","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":210,"title":["Epidemiology of systemic mastocytosis in Denmark"],"prefix":"10.1111","volume":"166","author":[{"given":"Sarah S.","family":"Cohen","sequence":"first","affiliation":[{"name":"EpidStat Institute Ann Arbor MI USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stine","family":"Skovbo","sequence":"additional","affiliation":[{"name":"Department of Clinical Epidemiology Aarhus University Aarhus Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanne","family":"Vestergaard","sequence":"additional","affiliation":[{"name":"Mastocytosis Centre Odense University Hospital (MastOUH) Odense Denmark"},{"name":"Department of Hematology Odense University Hospital Odense Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Kristensen","sequence":"additional","affiliation":[{"name":"Mastocytosis Centre Odense University Hospital (MastOUH) Odense Denmark"},{"name":"Department of Pathology Odense University Hospital Odense Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"M\u00f8ller","sequence":"additional","affiliation":[{"name":"Mastocytosis Centre Odense University Hospital (MastOUH) Odense Denmark"},{"name":"Department of Pathology Odense University Hospital Odense Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carsten","family":"Bindslev\u2010Jensen","sequence":"additional","affiliation":[{"name":"Mastocytosis Centre Odense University Hospital (MastOUH) Odense Denmark"},{"name":"Department of Dermatology and Allergy Centre Odense University Hospital Odense Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jon P.","family":"Fryzek","sequence":"additional","affiliation":[{"name":"EpidStat Institute Ann Arbor MI USA"},{"name":"Department of Clinical Epidemiology Aarhus University Aarhus Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sigurd","family":"Broesby\u2010Olsen","sequence":"additional","affiliation":[{"name":"Mastocytosis Centre Odense University Hospital (MastOUH) Odense Denmark"},{"name":"Department of Dermatology and Allergy Centre Odense University Hospital Odense Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,4,25]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0021-9681(87)90171-8"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2012.10.015"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2009.05.003"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.287.5462.2398"},{"key":"e_1_2_6_6_1","first-page":"54","volume-title":"World Health Organization (WHO) Classification of Tumours: Pathology & Genetics, Vol. 2 Tumours of Haematopoietic and Lymphoid Tissues","author":"Horny 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