Current Status of Hemostatic Treatment for Patients with Hemophilia and Inhibitors in Overseas Guidelines

Towards the Establishment of a Domestic Guideline

Abstract

The primary approach to the hemostatic management of patients with hemophilia and inhibitors includes treatment with bypassing agents or inhibitor neutralization with high doses of factor VIII (IX) concentrates. The choice of treatment for acute bleeding or surgery in inhibitor patients is dictated by the severity of the bleed, the current inhibitor titer, and the previous anamnestic response to factor VIII (IX). In general, a certain consensus for the choice of treatment has been established in overseas guidelines. Inhibitor neutralization therapy is recommended in low responder patients with a current titer of less than 5 Bethesda units (BU) /ml, while treatment with bypassing agents is recommended in high responders with a current titer of more than 5 BU/ml as the first-line therapy. In high responders with a current titer of less than 5 BU/ml, treatment with bypassing agents is chosen in minor bleeding, whereas the therapeutic choice in severe bleeding or surgery is controversial. Many of the guidelines do not fix an order of priority for the choice of bypassing agent, except for the recommendation of recombinant activated factor VII concentrates on the Australian guideline. These issues have been reviewed in this article for development of a domestic guideline for the treatment of patients with hemophilia and inhibitors.