Hematologic aspects of systemic mastocytosis

Systemic mast cell disease (SMCD), unlike cutaneous mast cell disorders, is characterized by mast cell infiltration of extracutaneous organs. Typically, this process involves mast cell infiltration of the bone marrow, frequently with consequent hematologic abnormalities. These abnormalities can be categorized as involving bone marrow histology, peripheral blood abnormalities, and functional hematologic abnormalities (i.e., coagulopathies) resulting from mast cell infiltration. Systemic mast cell disease has also been described as occurring in conjunction with a variety of hematologic malignancies. This article discusses each of these hematologic abnormalities and comments on the clinical significance or, where appropriate, the medical management of these findings.