Mastocytosis (International Archives of Allergy and Immunology, Vol 127, No. 2,2002)

Description

Since their discovery by Paul Ehrlich and their delineation as distinct hematopoietic cells, mast cells have become an ever-important subject of biomedical research. Apart from their well-known functions in allergic diseases, mast cells can also undergo neoplastic transformation. Intriguing examples are aggressive mastocytosis and mast cell leukemia. Historically, however, mastocytosis was first described in 1869 as a benign disease confined to the skin, and it was not until 1949 that a systemic form of mastocytosis was reported in an autopsy case. During the last few decades it was found that systemic mastocytosis can show an indolent, aggressive or even leukemic clinical course. More recently, a number of cell-specific and/or disease-related markers have been identified. Among them are lineage-associated transcription factors, growth factor receptors, disease-specific mutations and surface marker antigens. In addition, a number of prognostic markers have been proposed.

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