Myeloid proliferation associated with Down syndrome: a case report

Abstract

Background. Atypical chronic myeloid leukemia (aCML), BCR-ABL1 negative is a rare myelodysplastic syndrome (MDS)/myeloproliferative neoplasm (MPN) for which no current standard of care exists. ACML is characterized by many clinical features (splenomegaly, myeloid predominance in the bone marrow with some dysplastic features but without a differentiation block) and laboratory abnormalities (myeloid proliferation, low leukocyte alkaline phosphate values). A review of the literature suggests that the presence of an abnormal chromosome 21 may predispose to the development of leukemia.