Acquired coagulation factor inhibitors

Abstract

This chapter describes acquired coagulation factor inhibitors. These are autoantibodies directed against native clotting factor in persons without an underlying bleeding disorder. It is a rare disorder, but can result in life-threatening hemorrhage which can be difficult to manage. The inhibitors for the following factors are discussed in this chapter: Factor VIII (FVIII), von Willebrand Factor (VWF), Factor V (FV), prothrombin and thrombin. The inhibitors of FVIII are autoantibodies that bind to native FVIII in a person without congenital hemophilia and antibody binding leads to FVIII deficiency. The severity of bleeding is quite variable and approximately one-third of patients will have only minor bleeding that does not require any treatment and the most common sites of bleeding occurs in subcutaneous tissue, muscle and gastrointestinal tract. Two major aspects of management of acquired FVIII inhibitors include treatment of acute bleeding and eradication of the inhibitor and treatment options available are DDAVP, FVIII infusions and bypassing agents.