Antithrombotic therapy in patients with inherited bleeding disorders: practical considerations

Area of Science:

• Hematology
• Cardiology
• Vascular Medicine

Background:

• Inherited bleeding disorders like hemophilia were long thought to protect against thromboembolism (TE).
• This perception stemmed from low life expectancy and lack of observed TE in people with hemophilia (PWH) historically.
• Advances in hemophilia management have increased life expectancy, leading to the recognition of TE events in PWH.

Purpose of the Study:

• To review and provide guidance on managing arterial and venous TE in individuals with inherited bleeding disorders.
• To discuss the safety and efficacy of anticoagulant therapies in the context of hemophilia management.

Main Methods:

• Review of current literature on thromboembolism in inherited bleeding disorders.
• Analysis of anticoagulant therapy safety based on clotting factor levels (Factor VIII or IX > 20 IU/dL).
• Evaluation of alternative antithrombotic strategies to minimize exposure.

Main Results:

• Anticoagulant therapy should be used cautiously in severe/moderate hemophilia without prophylaxis.
• Therapeutic anticoagulation is safer when Factor VIII or IX levels are consistently ≥ 20 IU/dL.
• Direct oral anticoagulants are preferred for stroke prevention and venous TE treatment in PWH.

Conclusions:

• Hemophilia patients are at risk for thromboembolic events.
• Management requires careful balancing of anticoagulation risks and benefits.
• Alternative strategies like cardioversion, ablation, LAA occlusion, and drug-eluting stents can minimize antithrombotic exposure.