Lupus erythematosus complicated by hemophagocytic syndrome.

We report the case of a 28-year-old woman with systemic lupus erythematosus who, during a flare, suddenly developed febrile pancytopenia, hyperferritinemia, and abnormal liver function tests. Bone marrow aspiration confirmed hemophagocytic syndrome (HPS). Lupus-related HPS was diagnosed and the patient was treated with intravenous immunoglobulin, high-dose steroids, and cyclophosphamide with an excellent outcome. In febrile patients with lupus, pancytopenia together with very high ferritin levels should raise the suspicion of HPS and because this may be fatal, early bone marrow aspiration is mandatory for the diagnosis. The exclusion of concurrent infection as the cause of HPS is very important for the establishment of the right therapeutic strategy.