A 53-year-old man was admitted with pancytopenia, fever and splenomegaly. Biochemistry showed increased ferritin levels. Bone marrow examination revealed increased erythrocytic precursors (94.9%) and active hemophagocytosis. Pure erythroid leukemia with hemophagocytic syndrome (HPS) was diagnosed. Induction chemotherapy comprising idarubicin and cytarabine was administered and steroid pulse therapy was added. Complete remission was attained, and HPS also improved. However, leukemia relapsed during chemotherapy and the patient died. This is the first report of pure erythroid leukemia complicated with HPS.
2009 by The Japanese Society of Internal Medicine