Epidemiology of systemic mastocytosis in Denmark

• DOI: 10.1111/bjh.12916
• Publication Date: 2014-04-25T06:52:49Z
• Authors: Sarah S. Cohen; Stine Skovbo; Hanne Vestergaard; Thomas Kristensen; Michael Møller; Carsten Bindslev‐...; Jon P. Fryzek; Sigurd Broesby‐Olsen
• Keywords: Urticaria pigmentosa; Cutaneous Mastocytosis; Cumulative incidence; Danish

Summary Mastocytosis is a heterogeneous group of diseases characterized by abnormal proliferation mast cells. Systemic mastocytosis ( SM ), in which cells are present tissues beyond the skin, divided into seven subcategories with varying degrees severity and prognosis. Very little known about epidemiology its subcategories. This retrospective cohort study 548 adults diagnosed 1997–2010 was constructed using linked Danish national health registries. The most common subtype indolent (including urticaria pigmentosa) n = 450; 82%), followed unknown 61; 11%), associated clonal haematological non‐mast cell lineage disease 24; 4%), aggressive 8; 2%), leukaemia 5; 1%). incidence rate for (all subtypes including 0·89 per 100 000 year. Cumulative 12·46 000, 14‐year limited‐duration prevalence as 1 January, 2011 9·59 000. nationwide from Denmark first population‐based epidemiological mastocytosis. In this patients aged 15 years older, found to be overall relatively rare notable variation patient characteristics, survival measures.