Von Willebrand Disease
Von Willebrand disease is an inherited quantitative or qualitative defect of von Willebrand factor, impairing platelet adhesion and factor VIII stability and causing predominantly mucocutaneous bleeding; treatment depends on the subtype, bleeding phenotype, procedure and a documented response plan.
Definition
Von Willebrand disease is an inherited quantitative deficiency or qualitative defect of von Willebrand factor, a protein that supports platelet adhesion at injured vessels and stabilises factor VIII. It causes a predominantly mucocutaneous and procedure-related bleeding disorder, with severity ranging from mild type 1 disease to severe type 3 disease.
First principles
Von Willebrand factor links primary haemostasis and factor VIII
Von Willebrand factor binds exposed vessel-wall collagen and tethers platelets through platelet glycoprotein Ib under shear, while also carrying and stabilising factor VIII in plasma. Deficiency or dysfunction therefore produces platelet-type mucosal bleeding and, when factor VIII is sufficiently reduced, a secondary coagulation-factor phenotype.
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