Highlights & Basics
- Autosomal inheritance with variable penetrance and phenotypic expression.
- Usually presents with mucocutaneous bleeding.
- Menorrhagia and postpartum hemorrhage common in affected females.
- Joint bleeding rare and seen only in patients with more severe disease.
- Most patients have type 1 von Willebrand disease; more severe symptoms are seen in types 2 and 3.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
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]
Tosetto A, Rodeghiero F, Castaman G, et al. A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 vWD). J Thromb Haemost. 2006 Apr;4(4):766-73.[Abstract][Full Text]
Keeling D, Tait C, Makris M. Guideline on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders: a United Kingdom Haemophilia Center Doctors' Organisation (UKHCDO) guideline approved by the British Committee for Standards in Haematology. Haemophilia. 2008 Jul;14(4):671-84.[Abstract][Full Text]
1. 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]
2. 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]
3. Federici AB, Mannucci PM. Management of inherited von Willebrand disease in 2007. Ann Med. 2007;39(5):346-58.[Abstract]
4. Miller CH, Haff E, Platt SJ, et al. Measurement of von Willebrand factor activity: relative effects of ABO blood type and race. J Thromb Haemost. 2003 Oct;1(10):2191-7.[Abstract]
5. Castaman G, Federici AB, Rodeghiero F, et al. Von Willebrand's disease in the year 2003: towards the complete identification of gene defects for correct diagnosis and treatment. Haematologica. 2003;88:94-108.[Abstract][Full Text]
6. Srivastava A, Rodeghiero F. Epidemiology of von Willebrand disease in developing countries. Sem Thromb Hemost. 2005 Nov;31(5):569-76.[Abstract]
7. Peake I, Goodeve A. Type 1 von Willebrand disease. J Thromb Haemost. 2007 Jul;5 Suppl 1:7-11.[Abstract]
8. Blomback M, Konkle BA, Manco-Johnson MJ, et al. Preanalytical conditions that affect coagulation testing, including hormonal status and therapy. J Thromb Haemost. 2007;5:855-858.[Abstract]
9. Kumar S, Pruthi RK, Nichols WL. Acquired von Willebrand disease. Mayo Clin Proc.2002 Feb;77(2):181-7.[Abstract]
10. Ziv O, Ragni MV. Bleeding manifestations in males with von Willebrand disease. Haemophilia. 2004 Mar;10(2):162-8.[Abstract]
11. Kessler CM. Diagnosis and treatment of von Willebrand disease: new perspectives and nuances. Haemophilia. 2007;13(suppl 5):3-14.[Abstract]
12. Tosetto A, Rodeghiero F, Castaman G, et al. A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 vWD). J Thromb Haemost. 2006 Apr;4(4):766-73.[Abstract][Full Text]
13. Fressinaud E, Veyradier A, Truchaud F, et al. Screening for von Willebrand disease with a new analyzer using high shear stress: a study of 60 cases. Blood. 1998 Feb 15;91(4):1325-31.[Abstract][Full Text]
14. Hayward CP, Harrison P, Cattaneo M, et al. Platelet function analyzer PFA-100 closure time in the evaluation of platelet disorders and platelet function. J Thromb Haemost. 2006 Feb;4(2):312-9.[Abstract][Full Text]
15. Bodó I, Eikenboom J, Montgomery R, et al; Subcommittee on von Willebrand factor. Platelet-dependent von Willebrand factor activity. Nomenclature and methodology: communication from the SSC of the ISTH. J Thromb Haemost. 2015 Jul;13(7):1345-50.[Abstract]
16. Warner PE, Critchley HO, Lumsden MA, et al. Menorrhagia I: measured blood loss, clinical features and outcome in women with heavy periods: a survey with follow up data. Am J Obstet Gynecol. 2004 May;190(5):1216-23.[Abstract]
17. James AH, Kouides PA, Abdul-Kadir R, et al. Von Willebrand disease and other bleeding disorders in women: consensus on diagnosis and management from an international expert panel. Am J Obstet Gynecol. 2009 Jul;201(1):12.e1-8.[Abstract][Full Text]
18. 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]
19. Blomback M, Konkle BA, Manco-Johnson MJ, et al. Preanalytical conditions that affect coagulation testing, including hormonal status and therapy. J Thromb Haemost. 2007 Apr;5(4):855-8.[Abstract]
20. Nichols WL, Hultin MB, James AH, et al. Von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (US). Haemophilia. 2008;14:171-232.[Abstract][Full Text]
21. Rodeghiero F, Castaman G. Treatment of von Willebrand disease. Semin Hematol. 2005;42:29-35.[Abstract]
22. Keeling D, Tait C, Makris M. Guideline on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders: a United Kingdom Haemophilia Center Doctors' Organisation (UKHCDO) guideline approved by the British Committee for Standards in Haematology. Haemophilia. 2008 Jul;14(4):671-84.[Abstract][Full Text]
23. Rodeghiero F. Management of menorrhagia in women with inherited bleeding disorders: general principles and use of desmopressin. Haemophilia. 2008;14(suppl 1):21-30.[Abstract]
24. Sánchez-Luceros A, Meschengieser SS, Woods AI, et al. Biological and clinical response to desmopressin (DDAVP) in a retrospective cohort study of children with low von Willebrand factor levels and bleeding history. Thromb Haemost. 2010 Nov;104(5):984-9.[Abstract]
25. Ray S, Ray A. Non‐surgical interventions for treating heavy menstrual bleeding (menorrhagia) in women with bleeding disorders. Cochrane Database Syst Rev. 2016;(11):CD010338.[Full Text]
26. Coppola A, Franchini M, Makris M, et al. Thrombotic adverse events to coagulation factor concentrates for treatment of patients with haemophilia and von Willebrand disease: a systematic review of prospective studies. Haemophilia. 2012 May;18(3):e173-87.[Abstract]
27. Trigg DE, Stergiotou I, Peitsidis P, et al. A systematic review: the use of desmopressin for treatment and prophylaxis of bleeding disorders in pregnancy. Haemophilia. 2012;18:25-33.[Abstract][Full Text]
28. Demers C, Derzko C, David M, et al. Gynaecological and obstetric management of women with inherited bleeding disorders. J Obstet Gynaecol Can. 2005 Jul;27(7):707-32.[Abstract][Full Text]
29. Lee CA, Chi C, Pavord R, et al. The obstetric and gynaecological management of women with inherited bleeding disorders: review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors' Organization. Haemophilia. 2006 Jul;12(4):301-36.[Abstract][Full Text]
