Acquired haemophilia: experiences with a standardized approach

Acquired haemophilia is a rare, life-threatening, acquired bleeding diathes is. No general consensus exists on the best therapeutic approach. We report on the standardized approach at our institution evaluated in ten patients with acquired haemophilia. Factor VIII inhibitors were found in all patient s, activities ranging from 1 to 648 Bethesda units (BU). Eight of the ten p atients presented with severe bleeding. Two patients died during the acute phase, one from intracranial bleeding and one due to Mycoplasma pneumonia. One patient with mild bleeding was treated with immunosuppression alone. Tw o patients with factor VIII inhibitor activities below 5 BU were started on factor VIII concentrate therapy. Therapy was successful in one and was cha nged to recombinant human activated factor VII infusion (rFVIIa) in the oth er, owing to insufficient factor VIII recovery. Six patients with factor VI II inhibitor activities above 5 BU were started on activated prothrombin co mplex concentrate (APCC) therapy. APCC treatment was successful initially i n all six patients and was changed to rFVIIa infusion in one for rebleeding . One patient did not receive any specific therapy. Immunosuppression with prednisolone (2 mg kg(-1)) was begun in nine patients and was continued wit h cyclophosphamide (2 mg kg(-1)) in six. A complete remission of the acquir ed haemophilia was found in seven of the eight patients surviving the acute phase, one had ii partial remission. All patients with acquired haemophili a could be managed effectively following our standardized approach. Routine administration of immunosuppression was associated with high inhibitor eli mination rates.