Mastocytosis – pathogenesis, clinical manifestation and treatment

Abstract

The term mastocytosis designates a group of rare disorders characterized by typical skin lesions, frequently associated episodes of anaphylaxis, and clinical symptoms related to the release of various mediators. Dermatologists/allergists are frequently the first to establish the diagnosis. The condition is based on clonal mast cell proliferation, usually in the skin or bone marrow and only rarely in the gastrointestinal tract or other tissues. In general, mastocytosis has a good prognosis in terms of life expectancy. Rare variants – including mast cell leukemia, aggressive mastocytosis, and the exceedingly rare mast cell sarcoma – require cytoreductive therapy. In cases associated with hematological neoplasms, the prognosis depends on the underlying hematologic disorder.