Long-lasting remission and successful treatment of acquired factor VIII inhibitors using cyclophosphamide in a patient with systemic lupus erythematosus

Acquired deficiency of clotting factor VIII (FVIII) is a rare bleeding diat hesis seldom encountered in systemic lupus erythematosus (SLE). Reduction o f FVIII activity by autoantibodies can cause potentially life-threatening s ituations. Herein, an SLE patient with a positive lupus anticoagulant (LAC) test who abruptly developed metrorrhagia 4 yr after diagnosis is reported. Coagulation tests revealed FVIII activity reduced to 3% and a prolonged aP TT. FVIII inhibitor(s) were found to be as high as 3.0 Bethesda Units. Plas mapheresis, immunoglobulins, prednisolone and FVIII plasma concentrates ind uced the cessation of metrorrhagia, but the clotting tests were barely impr oved. One month later, extensive ecchymosis appeared and worsened, despite re-administration of the previous therapy. Pulse cyclophosphamide followed by oral administration was then started with normalization of coagulation p arameters and long-lasting disease remission.