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Hemophagocytic syndrome associated with retinoic acid syndrome in acute promyelocytic leukemia

A 56‐year‐old woman with an acute promyelocytic leukemia (APL) developed a severe all‐trans‐retinoic (ATRA) syndrome on day 17 of treatment. Shortly after, she presented a picture of pancytopenia, hepatosplenomegaly, increased triglycerides, ferritin, and liver enzymes. A bone marrow biopsy showed abundant macrophages and no evidence of leukemia. Tests for secondary hemophagocytic syndrome (HPS) were negative. A diagnosis of HPS was made. Treatment with dexamethasone and high‐dose immunoglobulins was unsuccessful. Consolidation chemotherapy with idarubicin and ATRA rapidly reversed the HPS. The HPS in this patient could be related to the release of macrophage‐stimulating cytokines by APL cells during ATRA syndrome. Am. J. Hematol. 76:172–175, 2004. © 2004 Wiley‐Liss, Inc.