Abstract
A 7-year-old boy with severe hemophilia A and poor response to immune tolerance induction (ITT) treat-ment for 33 months was treated with factor VIII/von Willebrand factor (FVIII/VWF) concentrate used as rescue ITI and followed by plasma derived factor VIII (pd.FVIII) concentrate. 1, 500 units (55 units/kg) of FVIII/VWF concen-trate were administered 3 times per week. After the initiation of rescue ITI, the titer of inhibitor decreased rapidly. Furthermore, the titer of inhibitor decreased to less than 5 BU/ml within eights months. We have judged that partial success was achieved in this case after rescue ITI because the titer of inhibitor remained under 3 BU/ml for more than five months (Current inhibitor titer is 0.6 BU/ml). The joints and muscular bleeding episodes decreased as well, and there was marked improvement in quality of life (QOL) for this patient. The results suggest that the rescue ITI with FVIII/VWF is effective. Notwithstanding, further study is necessary to identify clearly the factors that influence the results of rescue ITI.
