Abstract
Systemic mastocytosis (SM) is characterized by clonal expansion of mast cells (MCs) in different organs, usually in bone marrow (BM) and variably also in other tissues like skin and gastrointestinal tract.1-3 Clinical manifestations are heterogeneous and can include MC mediator symptoms and a dysfunction of various organ systems, such as cytopenia, hepatosplenomegaly, ascites, and weight loss. Most patients with SM carry the activating mutation KIT D816V. Typically, patients with SM are also characterized by elevated serum levels of tryptase, an enzyme produced by MCs, and aberrant expression of CD25 and/or CD2 on MCs in BM and possibly other tissues.
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