[Diagnosis and treatment of congenital and acquired hemophilia]

Abstract

In recent years, the treatment of congenital hemophilia is a transition period for treatment using extended half-life clotting factor formulations. Although the half-life of these formulations is longer than that of the standard formulations, the strength and rate of the effect remain unchanged. Therefore, it is thought that we can adapt to any kind of case if we treat various kinds of plans within the insurance adaptation. Furthermore, the factor VIII-mimetic bispecific antibody has also been currently developed as a therapeutic drug for hemophilia A. The antibody has a long half-life of approximately 30 days, is not neutralized by inhibitors, and can be subcutaneously administered. Acquired hemophilia A (AHA) is a disorder characterized by bleeding caused by autoantibodies that are developed against factor VIII. In recent years, the diagnostic method and treatment for AHA have undergone no major change. The major symptoms of AHA-subcutaneous bleeding and muscle hemorrhage-differ from those of congenital hemophilia. In AHA, PT and VWF activities are normal, APTT is extended, factor VIII activity is decreased, and factor VIII inhibitor is positive. Bypassing agents are the mainstay of hemostasis treatment, and immunosuppressive therapy is essential for inhibitor eradication.

Keywords: Acquired hemophilia; Factor VIII inhibitor; Hemophilia.