2025 ASH ISTH NBDF WFH monitoring report on the 2021 clinical guidelines on the diagnosis and management of von Willebrand disease.

Abstract

Abstract: The American Society of Hematology (ASH), International Society on Thrombosis and Haemostasis (ISTH), National Hemophilia Foundation (NHF; now National Bleeding Disorders Foundation), and World Federation of Hemophilia (WFH) 2021 guidelines on the diagnosis and management of von Willebrand disease (VWD) included 11 recommendations on the diagnosis of VWD and 12 recommendations on the management of VWD, the most common inherited bleeding disorder. We describe the results of a review of the 2021 guidelines by the clinical co-chairs of the guideline panels requested by ASH to inform decision-making about the need for and timing of a guideline revision. An updated MEDLINE and Embase search applied the same terms as the ASH ISTH NHF WFH 2021 guidelines limited to studies from 2020 to 30 July 2024 (diagnosis) or 24 July 2024 (management). For the diagnosis of VWD, 432 studies were identified and underwent title and abstract review, with 17 undergoing full text review, and for the management of VWD, 288 studies were identified and underwent title and abstract review, with 37 undergoing full text review, to determine whether the data would change the strength or directionality of the existing recommendation or merit development of a new recommendation. Based on this review, the clinical co-chairs noted that none of the reviewed studies would change the direction or strength of the existing guideline recommendations. There will be continued monitoring of the ASH ISTH NHF WFH 2021 guidelines on the diagnosis and management of VWD to evaluate whether there is sufficient new evidence to warrant additional revisions.