Hemophilia B (Factor IX Deficiency)

The biology of factor IX deficiency leading to hemophilia B has important distinctions from factor VIII deficiency that leads to hemophilia A. In this article, the authors explore the unique biology of factor IX in hemostasis, including the importance of FIX distribution to the extravascular space and the implications on dosing of factor concentrates. The authors review basic treatment principles of hemophilia B, including extended half-life products, and highlight areas of ongoing therapeutic innovation for hemophilia B prophylaxis.