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Immunol Allergy Clin North Am. 2014 May;34(2):283-95. doi: 10.1016/j.iac.2014.01.003.

Epidemiology, prognosis, and risk factors in mastocytosis.

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Department of Dermatology and Allergy Biederstein, Technische Universität München, Biedersteiner Strasse 29, Munich 80802, Germany. Electronic address:


This article updates current knowledge about epidemiology, prognosis, and risk factors for major complications in mastocytosis. A prevalence of mastocytosis of 1 in 10000 inhabitants has been reported, but underdiagnosis is assumed. The prognosis for cutaneous and indolent systemic mastocytosis is excellent. For more advanced forms of disease, prognostic parameters have been identified. A high extent of skin involvement, increased basal serum tryptase values, and extensive blistering are risk factors for severe mast cell activation episodes in children, whereas these associations seem to be less strong or nonexistent for anaphylaxis and osteoporosis in adult patients with indolent systemic mastocytosis.


Epidemiology; Mast cell activity syndrome; Mastocytosis; Monoclonal mast cell activation syndrome; Prognosis; Risk factors

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