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systemic mast cell disease


Definition: a heterogeneous group of diseases with four major divisions: indolent mastocystosis, the most common, which involves skin (urticaria pigmentosa), marrow, and the gastrointestinal tract; mastocytosis with an associated hematologic disorder (mast cell infiltrates in marrow, spleen, liver, and lymph nodes); mast cell leukemia (immature mast cells infiltrating marrow, peripheral blood, and extramedullary tissues); and aggressive mastocytosis (accumulation of mast cells in various organs, release of mast cell mediators producing a clinical sign).

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.