Development and desensitization therapy of high-response factor VIIIinhibitors with severe allergic reaction in a moderate hemophilia Apatient

摘要

Neutralizing antibodies (inhibitors) against factor VIII/IX (FVIII/FIX) poses aserious and challenging complication in the hemophilia treatment. Allergicreaction is more common in hemophilia B and always companion with FIXinhibitors, but it is rare in hemophilia A (HA). So far only few casesdemonstrated FVIII-specific allergic response in hemophilia A. Coexistence ofallergic reactions with inhibitors was contraindicated for immune toleranceinduction (ITI) regimen which is the only proven therapy to eliminate inhibitor.We report a rare case of a 11-year-old boy with moderate HA who developed hightiter inhibitor and severe allergic reaction to both plasma derived andrecombinant FVIII concentrates. Inhibitor was eliminated with the use ofprednisone. Further desensitization protocol by administering rFVIII ofincreasing does from 0.01 IU/kg to 40 IU/kg with a pre-determined time scheduleallowed patient tolerance to the normal dose and infusion time to FVIII.