Acute megakaryoblastic leukemia associated with Sweet's syndrome, including review of the literature

A case of Sweet syndrome (SS) associated with acute megakaryoblastic leukemia (AMKL) is reported. A 66-year-old male was admitted to Ashikaga Red Cross Hospital because of skin eruption and sore throat. His eruption was tender and erythema-like nature. He developed a spiking fever after admission. CBC revealed pancytopenia, and a bone marrow specimen showed increased megakaryoblasts and fibrosis. These blasts were shown to be CD41-positive by flow cytometric analysis. A diagnosis of AMKL was then made. Skin biopsy revealed infiltration of neutrophils without vasculitis, compatible with SS. Oral administration of prednisolone was begun which improved his skin lesions considerably. He was then treated with low dose Ara-C, which was however ineffective. The blasts increased in the peripheral blood and he died on the 72nd hospital day. There are 37 reported cases of SS associated with acute nonlymphocytic leukemia, and this is the first case report of SS associated with AMKL.