Von Willebrand disease: A century of progress.

One hundred years after the initial description of von Willebrand disease, originally referred to as pseudohemophilia, this article is a tribute to Dr Erik von Willebrand and a testament to the progress in our understanding of von Willebrand factor. Main focuses have been on structure and hemostatic function, as well as the advancements in the diagnosis, genetics, and management of von Willebrand disease. Insightful early observations led to the discovery of the main VWF ligands and interaction domains and the molecular mechanisms controlling these associations in the context of hemostasis. Reflecting these intricate mechanisms, the genetics and diagnosis of von Willebrand disease remain challenging, especially for the mild, quantitative deficiencies. Treatment developments and innovations have historically progressed quite slowly and in the shadow of hemophilia. However, recent patient-centered studies underscoring unmet clinical needs have catalyzed a dynamic and rapidly evolving effort to improve patient care and clinical outcomes.