systemic mast cell disease

systemic mast cell disease

a heterogeneous group of diseases with four major divisions: indolent mastocystosis, the most common, which involves skin (for example, urticaria pigmentosa), marrow, and the gastrointestinal tract; mastocytosis with an associated hematologic disorder (that is, mast cell infiltrates in marrow, spleen, liver, and lymph nodes); mast cell leukemia (that is, immature mast cells infiltrating marrow, peripheral blood, and extramedullary tissues); and aggressive mastocytosis (that is, accumulation of mast cells in various organs, release of mast cell mediators producing a clinical sign).
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A bone marrow biopsy demonstrated systemic mast cell disease involving 5 to 10% of a hypercellular bone marrow and a diagnosis of systemic mastocytosis was made.
Clinical, genetic, and therapeutic insights into systemic mast cell disease.
Flt-3 and c-kit mutation studies in a spectrum of chronic myeloid disorders including systemic mast cell disease.
The prevalence of systemic mast cell disease (MCD) is unknown, and the diagnosis is often elusive because serum and urine markers may become positive only after one of the self-limited, recurrent hypotensive episodes.
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