von Willebrand disease: an illustrated review

Department

Oncology and Hematology

Document Type

Article

Publication Title

Research and Practice in Thrombosis and Haemostasis

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.

First Page

106838

DOI

10.1016/j.rpth.2026.106838

Volume

10

Issue

5

Publication Date

7-1-2026

Medical Subject Headings

Humans; von Willebrand Diseases; von Willebrand Factor; Female; Hemostasis; Hemostatics; Pregnancy; Hemorrhage; Quality of Life

PubMed ID

42598104

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