How I treat patients with inherited bleeding disorders who need anticoagulant therapy

Abstract

Situations that ordinarily necessitate consideration of anticoagulation, such as arterial and venous thrombotic events and prevention of stroke in atrial fibrillation, become challenging in patients with inherited bleeding disorders such as hemophilia A, hemophilia B, and von Willebrand disease. There are no evidence-based guidelines to direct therapy in these patients, and management strategies that incorporate anticoagulation must weigh a treatment that carries a risk of hemorrhage in a patient who is already at heightened risk against the potential consequences of not treating the thrombotic event. In this paper, we review atherothrombotic disease, venous thrombotic disease, and atrial fibrillation in patients with inherited bleeding disorders, and discuss strategies for using anticoagulants in this population using cases to illustrate these considerations.

Figure 1

Recommended management algorithm for AF in hemophilia. CFC, clotting factor concentrate; CHADS₂, congestive heart failure, hypertension, age ≥75, diabetes, stroke/transient cerebral ischemia (doubled) score. Reprinted with permission from Mannucci et al.

Figure 2

Approach to FVIII or FIX goals for the use of anticoagulant and antiplatelet therapy in hemophilia. Red, avoid use; green, likely okay to use; peach, decisions on use made after considerations as outlined in the figure.