Blood Spotlight Cardiovascular risk in patients with hemophilia

The concept of risk factors in cardiovascular disease (CVD) has been well established. Smoking, hypertension, obesity, hypercholesterolemia, diabetes mellitus, and a positive family history for CVD are all associated with an increased risk of morbidity and mortality because of CVD. A prothrombotic state also contributes to the development of CVD. Increased levels of fibrinogen, von Willebrand factor (VWF), and factor VIII have all been linked to arterial thrombosis. VWF is essential for platelet adhesion and aggregation. Furthermore, VWF acts as the carrier protein for coagulation factor VIII. Factor VIII contributes to the formation of a fibrin-rich clot and also has a role in the formation of occluding thrombi in stenotic vessels. Several observational studies have suggested that patients with hemophilia A, who have a congenital deficiency of clotting factor VIII, may have a lower mortality because of arterial thrombosis compared with the general population. This protection may be due to hypocoagulability, which is associated with decreased thrombin generation and results in inhibition of thrombus formation. Recently, several studies found that hemophilia A patients, like the general population, have a high prevalence of atherosclerotic plaques. Furthermore, classical cardiovascular risk factors, such as hypertension, seem even more prevalent in hemophilia patients. Indeed, in clinical practice, an increasing number of hemophilia patients are diagnosed with CVD. The antithrombotic treatment of patients with a lifelong hypocoagulability and consequently a higher bleeding risk is a major challenge in clinical practice. In this review, we discuss recent developments of cardiovascular risk factors and atherosclerosis in patients with hemophilia.