Finally, (4) studies of patients with primary disorders of excess or deficiency of function in the vWF axis (e.g. thrombotic thrombocytopenic purpura and von Willebrand disease, respectively) which demonstrate the crucial role of vWF in atherothrombosis.
Interaction of von Willebrand factor (VWF) with platelet glycoprotein Ib (GPIb) and interaction of collagen with GPVI are essential for thrombus formation on ruptured or eroded atherosclerotic plaques (atherothrombosis).
Despite the high population impact of VWD and increasing knowledge of the pathophysiological role of VWF in atherothrombosis, data concerning atherosclerosis and its vascular complications in VWD patients are rather limited, and even more scarce when clinical management is considered.
Elucidating the details of VWF and GPIbalpha function will lead to a more satisfactory definition of the role of platelets in atherothrombosis, since hemodynamic forces greatly influence responses to vascular injury in stenosed and partially occluded arteries.