Successful treatment of high titer inhibitors in mild hemophilia a with avoidance of factor VIII and immunosuppressive therapy

We describe two patients with mild hemophilia A (MHA) who developed high ti ter inhibitor (HTI) following intensive recombinant factor VIII (rFVIII) co ncentrate replacement for surgery and trauma. Intranasal desmopressin was i nstituted shortly following Immunosuppressive therapy (IST) and activated p rothrombin complex concentrate (APCC) in one case, and following APCC alone in the second case. Avoidance of factor VIII (FVIII) coupled with intranas al desmopressin prophylaxis three times a week resulted In undetectable inh ibitor levels. Both patients have had no further bleeding episodes and have been maintained on desmopressin prophylaxis prior to activity for the past 2 to 3 years. Recombinant factor VIIa (rFVIIa) was used successfully prior to a second surgery In one patient without complication. (C) 2001 Wiley-Li ss, Inc.