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Epidemiology of systemic mastocytosis in Denmark
Author(s) -
Cohen Sarah S.,
Skovbo Stine,
Vestergaard Hanne,
Kristensen Thomas,
Møller Michael,
BindslevJensen Carsten,
Fryzek Jon P.,
BroesbyOlsen Sigurd
Publication year - 2014
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.12916
Subject(s) - epidemiology , systemic mastocytosis , urticaria pigmentosa , medicine , cutaneous mastocytosis , cohort , retrospective cohort study , incidence (geometry) , cohort study , cumulative incidence , population , danish , mast cell , disease , pediatrics , immunology , environmental health , linguistics , physics , philosophy , optics
Summary Mastocytosis is a heterogeneous group of diseases characterized by abnormal proliferation of mast cells. Systemic mastocytosis ( SM ), 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 SM and its subcategories. This retrospective cohort study of 548 adults with SM diagnosed 1997–2010 was constructed using linked Danish national health registries. The most common subtype of mastocytosis was indolent SM (including urticaria pigmentosa) ( n = 450; 82%), followed by SM with subtype unknown ( n = 61; 11%), SM with associated clonal haematological non‐mast cell lineage disease ( n = 24; 4%), aggressive SM ( n = 8; 2%), and mast cell leukaemia ( n = 5; 1%). The incidence rate for SM (all subtypes including urticaria pigmentosa) was 0·89 per 100 000 per year. Cumulative incidence was 12·46 per 100 000, and the 14‐year limited‐duration prevalence as of 1 January, 2011 was 9·59 per 100 000. This nationwide cohort from Denmark is the first population‐based epidemiological study of mastocytosis. In this cohort of patients aged 15 years and older, SM was found to be overall relatively rare with notable variation by subtype for patient characteristics, survival and epidemiological measures.