Do Hemophilia A and von Willebrand Disease Protect Against Carotid Atherosclerosis? A Comparative Study Between . Coagulopathics and Normal Subjects by Means of Carotid Echo-Color Doppler Scan

Abstract

Atherosclerosis is a multifactorial disease caused by genetic and environmental factors with important clinical sequelae. The aim of this study was to evaluate the degree of carotid atherosclerosis by echo-color Doppler scan in a group of patients affected by hemophilia A and von Willebrand disease versus a group of normal subjects apparently free of atherosclerotic risk factors. All coagulopathics and normal patients who came to our Internal Medicine Department (Padua Hospital) underwent physical exam, blood analysis, standard electrocardiogram, chest x-ray, echo-color Doppler scan, and a thorough history. We examined 156 subjects, 76 coagulopathics (46 men, 30 women) and 77 normals (37 men, 40 women). Coagulopathics were affected by hypertension in 28.9% of cases, diabetes mellitus in 6.5%, dislipidemia in 17.1%, smoke in 39.4%, and obesity in 36.8% (p < .05). Echo-color Doppler scan revealed carotid plaques in 27.2% of control patients versus 13.1% of coagulopathics (p < .05). Hemophilics and subjects with von Willebrand disease with a more serious illness had fewer plaques than those with lighter defects. Coagulopathics showed 23.6% of the plaques we revealed on the whole, versus 76.3% of control subjects (p < .01), with a lighter degree of stenosis (p < .01). Our data demonstrate that patients with hemophilia A and von Willebrand disease have fewer carotid plaques and a smaller degree of carotid stenosis than normal subjects of the same sex and age. These data seem to strengthen the hypothesis that blood coagulation defects may allow protection against carotid atherosclerosis and its sequelae.

Prevalence and risk factors for heart disease among males with hemophilia

“…Von Willebrand factor levels may also play an important modifying role [26]. It is well known that individuals with blood group O have lower levels of von Willebrand factor than those with other blood types and, in patients with hemophilia A, a shorter half-life of infused FVIII has been observed in patients with type-O blood [24].…”

Decreased Coagulability Has No Clinically Relevant Effect on Atherogenesis

“…In a recently published study, presence of advanced atherosclerotic lesions in the carotid artery between patients suffering from hemophilia A and von Willebrand disease were compared with healthy controls. 32 Results suggested considerable protection in the patients with a clotting-factor deficiency, even in those suffering from mild von Willebrand disease. However, in that study, the selection procedure and a general characteristic of the study groups (ie, age) were not documented.…”

Characteristics of Older Patient with Haemophilia

“…Two studies, one Dutch and the other Italian study with patients with haemophilia have reached the same conclusion (Rosendaal et al 1990;Bilora, 1999). This is most probably due to psychological changes induced by the presence of a chronic disease and the enforced sedentary life style.…”