Diagnosis and management of patients with von Willebrand's disease in Italy: an Expert Meeting Report

Abstract

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Von Willebrand factor (vWF) is an adhesive and multimeric glycoprotein that is crucial for the initial adhesion and aggregation of platelets at sites of vascular injury, particularly through larger multimers, and it binds coagulation factor VIII (FVIII) in plasma, protecting it from inactivation and clearance1. vWF is synthesised in vascular endothelial cells and megakaryocytes and is stored, respectively, in Weibel-Palade bodies and alpha-granules and secreted in plasma and the subendothelial extracellular matrix2,3.
Von Willebrand's disease (vWD) is the most common inherited bleeding disorder and is characterised by great heterogeneity. The current classification of vWD identifies partial or complete quantitative vWF defects (types 1 and 3) or qualitative vWF defects (type 2)4. [...]