Von Willebrand Disease in the Netherlands

Abstract Von Willebrand Disease (VWD) is the most common inherited bleeding disorder resulting in mucocutaneous bleeding, like epistaxis, oral cavity bleeding and menorrhagia. VWD is caused by reduced or dysfunctional von Willebrand Factor (VWF). VWF levels are highly variable between VWD patients and within a patient over time. Also the clinical expression of VWD is very heterogeneous with a large variability in bleeding frequency and severity. The overall aim of this thesis was to investigate the genotypic and phenotypic determinants of VWF levels and bleeding in patients with VWD.