Immunosuppressive Treatment in Haemophiliacs with Inhibitors to Factor VIII and Factor IX

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Abstract
Nine patients with severe hemophilia A and inhibitors (inhibitor levels between 0.1-5.8 U/ml) and 3 patients with severe hemophilia B and inhibitors (inhibitor levels between 0.1-11 U/ml) were treated on a total of 16 and 13 occasions, respectively, with a large dose of antigen (factor VIII or factor IX) and cyclophosphamide (10-15 mg/kg body wt i.v. initially and then 2-3 mg/kg body wt orally for 7-10 days) in connection with severe bleeding and surgery. All the patients did not respond to treatment with factor VIII or factor IX concentrate alone, and all except 1 had strong secondary antibody increases. In 6 of the patients with hemophilia A the treatment (11 occasions) had a satisfactory hemostatic effect and even permitted neurosurgery without bleeding complications. The inhibitor level remained at zero for 5-10 days, after which it gradually began to return towards its original level. In these cases it was possible to give factor VIII in amounts which neutralized the inhibitor and afterwards raised the factor VIII initially to at least 50%. In the 3 patients with hemophilia B treatment (13 occasions) was successful except on 1 occasion, and surgery was performed without abnormal bleeding. The factor IX level was initially raised to at least 50% except in the 1 failure. The inhibitor level remained at 0 for 12 days-3 mo., after which it gradually rose towards its original level. One patient was treated on 8 occasions.

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