Treatment of von Willebrand disease

Abstract

Treatment for von Willebrand disease (VWD), the most common inherited bleeding disorder, has advanced considerably since its initial description by Erik von Willebrand who first described a family in the Åland Islands with severe bleeding. Infusion of von Willebrand factor (VWF)–containing concentrates continues to serve as the foundation of management, in addition to desmopressin, hormonal therapies, and antifibrinolytic agents such as tranexamic acid. Recent advancements in VWD management are underscored by the publication of evidence-based international guidelines addressing critical aspects such as VWF prophylaxis, the use of anticoagulants and antiplatelet agents for cardiovascular disease, and comprehensive management during surgical as well as obstetric and gynecologic scenarios. This review provides an overview of modern treatment strategies, discusses management in special populations, and explores emerging therapies and future directions for improving VWD care.