Acquired factor VIII inhibitor as initial manifestation of collagen vascular disease: response to combination immunosuppression as first-line therapy.

Acquired hemophilia A is a rare disorder caused by the development of inhibitors to coagulation factor VIII. The case of a 74-year-old man who presented with spontaneous ecchymoses and was found to have acquired hemophilia A is reported. Bleeding was acutely controlled using recombinant activated factor VII. Combined modality immunosuppression was promptly initiated using intravenous methylprednisolone (later converted to oral prednisone), cyclophosphamide and rituximab. By day 17, inhibitor titer had decreased from 8.5 Bethesda units/mL to 1.3 units/mL and was subsequently undetectable; by day 26, factor VIII activity had increased from less than 1% to 42% and was greater than 100% by day 43. All treatment had concluded by day 40. Reported cases of the use of rituximab for acquired hemophilia in the elderly were reviewed. The use of rituximab as part of a combined immunosuppression regimen is an effective first-line approach to acquired hemophilia.