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Highlights & Basics
- Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Type 1 VWD is more common and less severe than type 2 and 3 VWD.
- VWD usually presents with mucocutaneous bleeding or bleeding after trauma or surgery. Heavy menstrual bleeding and postpartum hemorrhage are common in women with VWD. Joint bleeding is rare and usually only occurs in patients with severe disease (e.g., type 3 VWD).
- Bleeding assessment tools (BATs) can help identify patients with VWD and guide referral for diagnostic testing.
- Diagnosis requires blood tests to assess von Willebrand factor (VWF) antigen (VWF:Ag), platelet-dependent VWF activity (e.g., using VWF:GPIbM assay), and factor VIII coagulant activity (FVIII:C).
- Management involves preventing and treating bleeding symptoms based on the specific type of VWD. Treatment options include desmopressin, VWF-containing concentrate, and antifibrinolytic therapy.
Quick Reference
History & Exam
Key Factors
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Definition
Epidemiology
Etiology
Pathophysiology
Citations
Sadler JE, Budde U, Eikenboom JC, et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the subcommittee on von Willebrand factor. J Thromb Haemost. 2006 Oct;4(10):2103-14.[Abstract][Full Text]
Pavord S, Rayment R, Madan B, et al; on behalf of the Royal College of Obstetricians and Gynaecologists. Management of inherited bleeding disorders in pregnancy. Green-top guideline No 71 (joint with UKHCDO). BJOG. 2017 Jul;124(8):e193-263.[Abstract][Full Text]
James PD, Connell NT, Ameer B, et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv. 2021 Jan 12;5(1):280-300.[Abstract][Full Text]
Laffan MA, Lester W, O'Donnell JS, et al. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Br J Haematol. 2014 Nov;167(4):453-65.[Abstract][Full Text]
Platton S, Baker P, Bowyer A, et al. Guideline for laboratory diagnosis and monitoring of von Willebrand disease: a joint guideline from the United Kingdom Haemophilia Centre Doctors' Organisation and the British Society for Haematology. Br J Haematol. 2024 May;204(5):1714-31.[Abstract][Full Text]
Connell NT, Flood VH, Brignardello-Petersen R, et al. ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease. Blood Adv. 2021 Jan 12;5(1):301-25.[Abstract][Full Text]
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