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. 2026 Jul 11;10(5):106838. doi: 10.1016/j.rpth.2026.106838

von Willebrand disease: an illustrated review

Mouhamed Yazan Abou-Ismail 1,∗, Peter A Kouides 2, Paula D James 3, Nathan T Connell 4
PMCID: PMC13471213  PMID: 42598104

Abstract

First described 100 years ago, von Willebrand disease (VWD) is the most common inherited bleeding disorder, characterized by a quantitative or qualitative deficiency of von Willebrand factor (VWF), a large multimeric glycoprotein central to hemostasis. Despite its prevalence, VWD remains globally underdiagnosed. Accurate diagnosis requires thorough clinical assessment and specialized laboratory evaluation. Although international evidence-based guidelines have advanced care, significant diagnostic and management challenges remain. Management focuses on treating and preventing bleeding, ensuring periprocedural safety, and improving quality of life through individualized strategies and the use of various hemostatic agents and therapeutic strategies. Optimal management approaches in complex clinical scenarios remain challenging. In this comprehensive illustrated review, we cover the biology and physiology of VWF, along with the pathophysiology, diagnosis, and management of VWD. We review current clinical practice recommendations and provide practical guidance for challenging scenarios such as surgery, pregnancy, cardiovascular disease, aging, and acquired von Willebrand syndrome. We also highlight emerging therapeutics and models of care in VWD.

Keywords: desmopressin; hemorrhage; hemostasis; hemostasis, surgical; menorrhagia; physiology; physiopathology; postpartum hemorrhage; postoperative hemorrhage; tranexamic acid; von Willebrand disease; von Willebrand factor

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Acknowledgments

Created using BioRender.com. All photographic images were used with permission. No generative artificial intelligence tools were used in the creation of the illustrations or text.

Funding

The authors received no funding for this study.

Author contributions

M.Y.A.I. illustrated all figures, designed all diagrams, conceived and conceptualized the review, and wrote the manuscript. N.T.C., P.A.K., and P.D.J. reviewed the outline and manuscript and provided critical input.

Relationship Disclosure

MYAI reports consulting or advisory for Genentech, Sanofi, and Takeda Pharmaceuticals USA. NTC reports serving as a consultant for OctaPharma AG and Takeda, participating in advisory boards for Bayer, CSL Behring, Genentech, Medzown, OctaPharma AG, SeraGene, and Takeda; holding equity in Medzown and Doximity, and receiving honoraria/travel support from CSL Behring, OctaPharma AG, and Roche. PDJ reports consulting for Star/Vega, Roche, BioMarin and honoraria from CSL Behring. PAK declares no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Footnotes

Handling Editor: Professor Michael Makris

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