A patient with a spontaneous factor VIII:C autoantibody: successful treatment with cyclosporine

A 65-year-old man was admitted to the hospital because of extensive spontaneous ecchymoses of the trunk and huge hematomas of the arms and legs. He had no personal or family history of a hemorrhagic diathesis. Coagulation studies revealed a prolonged APTT, no detectable factor VIII:C activity, and a high titer of anti-factor VIII:C antibodies. A diagnosis of acquired hemophilia was made. No underlying disorder could be found. The inhibitor was an IgG antibody. Long-term management of bleeding including immunodepletion by plasma exchange and immunosuppression by corticosteroids and cytotoxic drugs alone and in combination had no effect on the bleeding tendency and coagulation data. The administration of Cs (10 mg/kg/d) in combination with prednisone induced a remission. After a stormy course and a 5-month stay in the hospital the patient could be discharged. A relapse occurred after the Cs and prednisone dosages were reduced. Increasing the Cs dosage induced a remission again.