Blood Adv
ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease.
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.
Related Questions
How do you approach delivery planning in patients with T1 vWD?
For VWD, I recommend that the mode and timing of delivery be obstetric-based (i.e., the presence of VWD has no effect on the decision). If a CSX is needed, I consider an epidural safe if preceded by VWD-specific therapy. I base the duration of VWD-specific therapy on clinical history and 3rd-trimest...
How would you manage anti-platelet therapy in patients presenting with ischemic stroke and have a history of von Willebrand disease?
I follow the ASH ISTH NHF WFH 2021 guidelines, recommendation 3: "In patients with VWD and cardiovascular disease who require treatment with antiplatelet agents or anticoagulant therapy, the panel suggests giving the necessary antiplatelet or anticoagulant therapy over no treatment (conditional reco...