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T-Cell/Myeloid Mixed-Phenotype Acute Leukemia with KMT2A Rearrangement

Zivanai Cuthbert Chapanduka

Mixed Phenotype Acute Leukemia (MPAL) is a rare leukemia subtype arising from hematopoietic pluripotent stem cells. The hallmark of the disease is co-expression of myeloid antigens and B- or T-lymphoid antigens. We discuss an 11-year-old female, who presented with gum hypertrophy, lymphadenopathy and anemia with 84% blasts on peripheral blood examination. Immunophenotyping revealed two blast populations co-expressing markers of both T-cell and myeloid lineages with monocytic differentiation. Cytogenetics showed t (6;11). The diagnosis of T-cell/myeloid MPAL with KMT2A rearrangement was made. Two rare features noted were, the two distinct myeloid and monocytic blast sub-populations and T-cell/myeloid marker co-expression with KMT2A-rearrangement. The KMT2A rearrangement has been associated with B-cell/myeloid mixed phenotypic leukemia however association with T-cell/myeloid mixed phenotype is rare.