Recombinant factor VIIa in the management of a pseudotumour in acquired haemophilia

Bleeding in nonhaemophilic patients with high-titre factor VIII autoantibodies is often severe, life-threatening and refractory to treatment with factor VIII concentrates. In this report, we describe an elderly woman who required surgical excision of a large haemophilic pseudotumour adjacent to the left gluteal muscle. The Bethesda titre of 11 U precluded treatment with human factor VIII, and the patient had an anaphylactic reaction to porcine factor VIII. However, haemostasis was successfully achieved with recombinant factor VIIa and the pseudotumour was removed. The patient was treated with repeated cycles of cyclophosphamide, vincristine, and prednisone. The Bethesda titre eventually declined to 0.7 U and the factor VIII rose to 20%. During an 18-month follow-up period there has been no recurrence of bleeding or of the pseudotumour.