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Mast cell leukemia: a review


Mast cell leukemia (MCL) is a rare subtype of systemic mastocytosis. In MCL the numbers of mast cells exceed 19% of nucleated cells in bone marrow and/or 10% of circulating leukocytes in peripheral blood. Primary MCL must be distinguished from secondary MCL evolving from another subvariant of systemic mastocytosis or from mast cell sarcoma. Acute MCL with a poor prognosis is distinguished from the more indolent chronic MCL. Serum tryptase is significantly elevated in almost all MCL patients and activating point mutations at codon 816 of KIT (usually KIT D816V) are encountered in about 70%. Regarding differential diagnosis, other ‘tryptase-positive’ or ‘metachromatic’ leukemias must be considered, including myelomastocytic leukemia and tryptase-positive acute myeloid leukemia but also acute and chronic basophilic leukemias.