Hemophilia A, von Willebrand Disease, and Atherosclerosis of Abdominal Aorta and Leg Arteries: Factor VIII and von Willebrand Factor Defects Appear to Protect Abdominal Aorta and Leg Arteries From Atherosclerosis

Abstract

We hypothesized that patients with hemophilia or von Willebrand disease might be protected from atherosclerosis because of their coagulation defect. We studied 40 subjects affected by these two coagulation diseases using echocolor Doppler of the abdominal aorta and leg arteries, and compared the results with those obtained in 40 control patients who were homogenous with study patients in terms of sex, age, and risk factors for atherosclerosis. The probands presented a lower number of plaques than the 40 control subjects in the aorta and in the leg arteries. The most serious hemophilic patients had fewer plaques than controls or than patients with mild hemophilia. Both hemophilia and von Willebrand disease seem to protect against atherosclerosis.

Coronary Artery Calcification in Hemophilia A

“…[17][18][19][20][21] Srámek et al 21 and Sartori et al 20 found no differences in IMT, whereas 3 other studies [17][18][19] did show a lower IMT, fewer atherosclerotic plaques, or both in hemophilia patients. In these studies, patients were relatively young; mean age ranged from 40 to 58 years.…”

“…Moreover, comparison groups were not well chosen and, in some, not well described. [17][18][19][20][21] In addition, confounding was not accounted for in the analyses. These factors make interpretation of the results difficult.…”

“…A major disadvantage in most studies is the heterogeneous study population, combining patients with severe, moderate, and mild hemophilia A or B or von Willebrand disease. 17,18,20,21 Clotting factors VIII and IX and von Willebrand factor play different roles in the development of atherosclerosis. 12,34 Combining these disorders in 1 study might result in masking a possible relationship between 1 of the disorders and atherosclerosis.…”

“…16 Conflicting results have also been reported by studies comparing intima-media thickness (IMT) between hemophilia patients and nonhemophilic subjects. [17][18][19][20][21] This could be due to heterogeneous study populations including patients with other bleeding disorders than hemophilia, low mean ages, not well chosen or not well defined comparison populations, and variability in tests and outcome parameters.…”

Stress repair mechanism activity explains inflammation and apoptosis

“…This explains why shear stress cannot explain atherosclerosis resistance in Down's Syndrome [576,577], hemophilia, von Willebrand coagulopathy [578], and patients treated with anticoagulants.…”