Role of von Willebrand factor in the haemostasis.

Abstract

von Willebrand factor (VWF) is an adhesive and multimeric glycoprotein that found its historical origin in 1924, when the Finnish physician Erik von Willebrand first reported a family with a serious hereditary bleeding affecting consanguineous families1. The proband was a five years old girl with severe bleeding since birth. Three sisters had died before the age of four, one living sister, aged three, also was severely affected. von Willebrand had thought that it was a disorder of platelet function or a vascular defect as a possible cause of the bleeding. Since the original observations by Erik von Willebrand, the disease has been extensively studied and it was shown in the mid 1950s that impaired haemostasis was because of lack or an abnormality of a plasmatic factor - the von Willebrand factor - necessary for normal hemostasis2,3.