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. 2021 Jan 12;5(1):301-325.
doi: 10.1182/bloodadvances.2020003264.

ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease

Nathan T Connell  1 , Veronica H Flood  2 , Romina Brignardello-Petersen  3 , Rezan Abdul-Kadir  4 , Alice Arapshian  5 , Susie Couper  6 , Jean M Grow  7 , Peter Kouides  8 , Michael Laffan  9 , Michelle Lavin  10 , Frank W G Leebeek  11 , Sarah H O'Brien  12 , Margareth C Ozelo  13 , Alberto Tosetto  14 , Angela C Weyand  15 , Paula D James  16 , Mohamad A Kalot  17 , Nedaa Husainat  17 , Reem A Mustafa  17
Affiliations

ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease

Nathan T Connell et al. Blood Adv. .

Abstract

Background: von Willebrand disease (VWD) is a common inherited bleeding disorder. Significant variability exists in management options offered to patients.

Objective: These evidence-based guidelines from the American Society of Hematology (ASH), the International Society on Thrombosis and Haemostasis (ISTH), the National Hemophilia Foundation (NHF), and the World Federation of Hemophilia (WFH) are intended to support patients, clinicians, and health care professionals in their decisions about management of VWD.

Methods: ASH, ISTH, NHF, and WFH formed a multidisciplinary guideline panel. Three patient representatives were included. The panel was balanced to minimize potential bias from conflicts of interest. The University of Kansas Outcomes and Implementation Research Unit and the McMaster Grading of Recommendations Assessment, Development and Evaluation (GRADE) Centre supported the guideline development process, including performing and updating systematic evidence reviews (through November 2019). The panel prioritized clinical questions and outcomes according to their importance to clinicians and patients. The panel used the GRADE approach, including GRADE Evidence-to-Decision frameworks, to assess evidence and make recommendations, which were subject to public comment.

Results: The panel agreed on 12 recommendations and outlined future research priorities.

Conclusions: These guidelines make key recommendations regarding prophylaxis for frequent recurrent bleeding, desmopressin trials to determine therapy, use of antiplatelet agents and anticoagulant therapy, target VWF and factor VIII activity levels for major surgery, strategies to reduce bleeding during minor surgery or invasive procedures, management options for heavy menstrual bleeding, management of VWD in the context of neuraxial anesthesia during labor and delivery, and management in the postpartum setting.

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Conflict of interest statement

Conflict-of-interest disclosure; and all authors were members of the guideline panel or members of the systematic review team or both. As such, they completed a disclosure-of-interest form, which was reviewed by ASH and is available as supplemental Files 2 and 3.

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