Clinical Review

von Willebrand Disease: Approach to Diagnosis and Management

An updated version of this article is available.


von Willebrand disease (VWD) is an inherited bleeding disorder caused by deficient or defective plasma von Willebrand factor (VWF). VWF is an adhesive multimeric plasma glycoprotein that performs 2 major functions in hemostasis: it mediates platelet adhesion to injured subendothelium via glycoprotein 1bα (GPIbα), and it binds and stabilizes factor VIII (FVIII) in circulation, protecting it from proteolytic degradation by enzymes. The current VWD classification recognizes 3 types. In order to understand the role of the numerous laboratory investigations as well as the classification of VWD, it is important to review the structure and function of the VWF subunit. Bleeding symptoms reflect the defect in primary hemostasis: mucocutaneous bleeding and excessive bleeding after surgery or trauma. Treatment focuses on increasing VWF levels with desmopressin (1-deamino-8-D-arginine vasopressin, DDAVP) or clotting factor concentrates containing both VWF and FVIII (VWF/FVIII concentrate). Nonspecific treatment options include antifibrinolytic agents (tranexamic acid) and hormone therapy (oral contraceptive pill).

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