Challenges in the management of women with type 2B von Willebrand disease during pregnancy and the postpartum period: evidence from literature and data from an international registry and physicians’ survey—communication from the Scientific and Standardization Committees of the International Society on Thrombosis and Haemostasis

• Published in: Journal of thrombosis and haemostasis Vol. 21; no. 1; pp. 154 - 163
• Main Authors: Miljic, Predrag, Noureldin, Abdelrahman, Lavin, Michelle, Kazi, Sajida, Sanchez-Luceros, Analia, James, Paula D., Othman, Maha
• Format: Journal Article
• Language: English
• Published: England Elsevier Inc 01.01.2023
• Subjects: Female; Hemostasis; Humans; postpartum hemorrhage; Postpartum Period; Pregnancy; ristocetin; thrombocytopathy; thrombocytopenia; Thrombosis; von Willebrand Disease, Type 2; von Willebrand Diseases - diagnosis; von Willebrand Diseases - therapy; von Willebrand Factor; postpartum hemorrhage; thrombocytopathy; ristocetin; thrombocytopenia; von Willebrand factor
• ISSN: 1538-7836; 1538-7836
• DOI: 10.1016/j.jtha.2022.10.019

Management of women with type 2B von Willebrand disease (VWD) during pregnancy is challenging because of dysfunctional von Willebrand factor (VWF) and the complexity resulting from discrepant VWF/factor VIII (VWF/FVIII) levels, impaired platelet-dependent VWF activity, progressive thrombocytopenia, and risks associated with the use of desmopressin. There is a lack of high-quality evidence to support clinical decision making. In this study, we examined the current diagnostic and management approaches and outcomes in women with VWD during pregnancy. Data were collected via 3 avenues: literature review, an international registry, and an international survey on physicians’ practices for the management of pregnancy in women with VWD. The registry and survey were supported by the International Society on Thrombosis and Haemostasis. Data on clinical and laboratory features, management and bleeding complications, and pregnancy outcomes of a total of 55 pregnancies from 49 women across the globe (literature: 35, registry: 20) and data reported by 112 physicians were analyzed. We describe the largest dataset on pregnancies in women with type 2B VWD available to date. The data highlight the following key issues: a) bleeding complications remain a concern in these patients, b) the target safe VWF level and the ideal monitoring approach are unknown, c) there is a wide range of hemostatic management practices in the type and timing of treatment, and d) physicians have diverse views on the mode of delivery and use of neuraxial anesthesia. We conclude that an international consensus and guidance are critically required for better care and improved outcomes in this patient cohort. •Management of women with type 2B von Willebrand disease during pregnancy is challenging.•High-quality evidence to guide clinical decision making and management is lacking.•Three data resources inform this study: literature review, a registry, and a physicians’ survey.•Bleeding remains a concern, management practices are diverse, and an international guidance is needed.