血友病におけるインヒビターの発生機序とその治療戦略

DOI 被引用文献6件 参考文献52件

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  • Mechanism of the Inhibitor Development and Its Management in Patients with Hemophilia.

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The development of coagulation factor VIII or factor IX inhibitor remains a major serious complication of treatment in patients with hemophilia. The incidence of the factor VIII inhibitor is 20-30%, according to the recent prospective studies. Major factor VIII gene defects, such as large deletion, intron 22 inversion, and nonsense mutation that result in no detectable circulating antigen, are potential risk factors. However, no exact explanation is available for why some hemophiliacs. develop inhibitors. The treatment of hemophiliacs with inhibitors are divided into two groups. One is the hemostatic treatment. Replacement therapy may be effective in some patients with a transient or low responder inhibitor. (Activated) prothrombin complex concentrates are mainly used in Japan, but long-term usage of the products is limited because of anamnestic response, thrombotic complications, and economic issues. Recombinant factor VII product was recently introduced to Europe and North America, and it is expected to be approved soon in Japan. The other is immunological therapy for the reduction of inhibitor. The most reliable immunological therapy is immune tolerance treatment (ITT). Overall, the efficacy of ITT is 60-80%. Several ITT trials have been conducted in Japan by using mainly moderate to low-dose protocol. The establishment of Japanese protocol of ITT is urgently needed.

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