100 Years of von Willebrand Disease

von Willebrand disease (VWD) is considered the most common inherited bleeding disorder, even surpassing haemophilia A. Nevertheless, VWD may be underdiagnosed, overdiagnosed, or misdiagnosed, depending on the expertise of the managing clinician and the testing laboratory. This is due to the heterogeneity of VWD and the complexities of laboratory assessment. For example, in haemophilia, there is a deficiency or defect in a single clotting factor, either factor VIII or factor IX, for haemophilia A and B, respectively. In contrast, at least six types can be identified in VWD, classified according to the defect and/or deficiency in the plasma protein von Willebrand factor. von Willebrand factor has numerous functions, most of which can be assessed by laboratory testing. However, unlike haemophilia, a battery of laboratory tests is required to enable a diagnosis or exclusion of VWD, as well as its type classification. This complexity is not well understood by many clinicians or scientists. VWD was first described by Erik von Willebrand in 1926, and so 2025 represents 100 years of VWD. The authors review some of the history of VWD, as well as outlining the current state of play for diagnosis and treatment.