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Successful treatment of reactive hemophagocytic syndrome by plasmapheresis and high-dose γ-globulin in a patient with systemic lupus erythematosus

Abstract A 31-year-old woman who had been administered corticosteroid and immunosuppressive agents for systemic lupus erythematosus (SLE) without flare-up was diagnosed as having reactive hemophagocytic syndrome (HPS) with severe disseminated intravascular coagulation. The causative underlying disease was uncertain, but it was not the SLE itself. Her fulminant HPS with increased serum ferritin and inflammatory cytokines (sIL-2R, TNF-α, IL-6, and IFN-γ) was successfully treated with plasmapheresis and high-dose γ-globulin therapy.