Synchronization of plasma exchange and cyclophosphamide in severe and refractory autoimmune hemolytic anemia

Abstract

Two cases of severe autoimmune hemolytic anemia (AIHA) that failed multiple treatment modalities obtained complete and long-lasting remissions with a combination of three one volume plasma exchange (PE) on succeeding days followed 6 hours later on the 3rd day by cyclophosphamide (cyc) 750 mg/m2 IV, and cyc/prednisone (pred) qd tapering to either no therapy or minimal therapy over a 6 month period. Both cases remain without evidence of AIHA after 43 and 19 months follow-up. Possible non-exclusive mechanisms that explain this favorable outcome are enhanced cytotoxic effect of cyc on proliferating lymphocytes participating in the antibody rebound phenomena, suppression of B lymphocytes with daily cyc/pred, and/or formation of anti-idiotype antibodies.