The American Society of Hematology (ASH) has developed clinical practice guidelines for the diagnosis and management of von Willebrand Disease (VWD) in partnership 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. The guidelines were published on January 12, 2021 in Blood Advances.
Methods:
ASH, ISTH, NHF, and WFH established a multidisciplinary guideline panel that included 4 patient representatives and was balanced to minimize potential bias from conflicts of interest. The Outcomes and Implementation Research Unit at the University of Kansas Medical Center (KUMC) supported the guideline-development process, including performing or updating systematic evidence reviews up to 8 January 2020. The panel prioritized clinical questions and outcomes according to their importance for clinicians and patients. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including GRADE Evidence-to-Decision frameworks, to assess evidence and make recommendations, which were subsequently subject to public comment.
The panel agreed on 11 recommendations.
Key recommendations of these guidelines include the role of bleeding-assessment tools in the assessment of patients suspected of VWD, diagnostic assays and laboratory cutoffs for type 1 and type 2 VWD, how to approach a type 1 VWD patient with normalized levels over time, and the role of genetic testing vs phenotypic assays for types 2B and 2N. Future critical research priorities are also identified.