Epidemiology of systemic mastocytosis in Denmark

Adult Male Urticaria pigmentosa Adolescent Epidemiology Denmark Middle Aged Systemic/epidemiology Denmark/epidemiology 3. Good health Young Adult 03 medical and health sciences 0302 clinical medicine Mastocytosis, Systemic Mastocytosis, Systemic/epidemiology Humans Female Epidemiologic Methods Mastocytosis Indolent systemic mastocytosis Aggressive systemic mastocytosis Aged
DOI: 10.1111/bjh.12916 Publication Date: 2014-04-25T06:52:49Z
ABSTRACT
SummaryMastocytosis 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.
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