Management of women with type 2B von Willebrand disease during pregnancy and postpartum: guidance from ISTH SSC subcommittees on von Willebrand factor and women's health issues in thrombosis and hemostasis

Type 2B von Willebrand disease (VWD) is a rare qualitative variant, accounting for ∼5% of all VWD cases. It is characterized by increased affinity of abnormal von Willebrand factor (VWF) for the platelet glycoprotein Ibα receptor, resulting in enhanced clearance of both high-molecular-weight VWF multimers and platelets from circulation. The management of women with type 2B VWD during pregnancy and the postpartum period poses unique challenges due to complex hemostatic abnormalities, a high risk of bleeding complications, and a lack of evidence-based guidelines. A recent systematic review, international registry analysis, and global physician survey highlighted several unmet clinical needs in this population, including gaps in early diagnosis, prenatal counseling, pregnancy monitoring, and peripartum management. In response, the International Society on Thrombosis and Haemostasis Scientific Subcommittees on VWF and on Women's Health Issues in Thrombosis and Haemostasis collaborated to develop consensus-based guidance for the management of type 2B VWD in pregnancy and postpartum. Using the real-time Delphi methodology, 14 international experts reviewed 26 initial statements on diagnosis, monitoring, and treatment. After 2 rounds of anonymous voting and revisions based on participants' feedback, consensus was achieved on 25 statements. These consensus statements, grounded in the best available evidence and expert opinion, aim to standardize care, guide management, and improve clinical outcomes for women with type 2B VWD during pregnancy and postpartum.