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A 16-month-old girl presented with hepatosplenomegaly and pancytopenia. Bone marrow aspiration showed a florid increase in macrophages with marked hemophagocytosis. She subsequently improved spontaneously with no therapeutic intervention, but 2 months later presented with frank acute monoblastic leukemia. This case illustrates the difficulties in classifying malignancies of the monocyte-macrophage lineage and how hemophagocytosis can be the presenting feature of a range of diseases.
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