Von Willebrand Disease (VWD) is the most common inherited bleeding disorder with wide variability in clinical phenotype. Arriving at an accurate diagnosis can be challenging due to variability in test accuracy, diagnostic thresholds, and access to specialty centers. Management of VWD may involve use of von Willebrand factor concentrate for the prevention and treatment of bleeding as well as adjunctive therapies such as desmopressin and antifibrinolytics. ASH has developed guidelines on diagnosis and management of VWD with the International Society of Thrombosis and Haemostasis (ISTH), National Hemophilia Foundation (NHF), World Federation of Hemophilia (WFH) and the University of Kansas Medical Center.
• Review methods for development of clinical practice guidelines
• Describe diagnostic pathway for evaluation of von Willebrand disease
• Discuss diagnostic thresholds and subtype classification of VWD
• Discuss utility of prophylaxis for patients with severe bleeding in VWD
• Review management approach for heavy menstrual bleeding and postpartum hemorrhage