Endothelial dysfunction in von Willebrand disease: angiogenesis and angiodysplasia

Abstract

In recent years, new functions for the haemostatic protein von Willebrand Factor (VWF) have emerged. Amongst these is the ability to modulate the development of new blood vessels, a process called angiogenesis. The subtle effects that VWF exerts on blood vessel formation and stability may be relevant for the small but significant fraction of patients with von Willebrand disease (VWD) who also present with vascular malformations (angiodysplasia) in the gastrointestinal tract, often responsible for intractable bleeding. This review will briefly summarise the evidence and discuss the molecular pathways involved.

Keywords: endothelial cells; extracellular matrix; vascular malformations; von willebrand factor; weibel palade bodies.