Abstract
Systemic mastocytosis (SM) is a heterogeneous group of disorders characterized by clonal mast cell expansion within the hematopoietic compartment and other tissues. Patients present with manifestations ranging from cutaneous to systemic symptoms relating to mast cell mediator release and/or tissue infiltration. SM is currently classified into one of several variants: indolent SM (ISM), smoldering SM, SM with associated hematological neoplasm, aggressive SM, and mast cell leukemia, with important implications relating to prognosis and management.
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