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Von Willebrand disease

Last updated: December 22, 2020

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Von Willebrand disease (vWD) is a bleeding disorder characterized by a deficiency or dysfunction of von Willebrand factor (vWF). In the vast majority of cases, vWD is an inherited disorder caused by mutations in the vWF gene. vWF is involved in platelet adhesion and prevents degradation of factor VIII. Therefore, vWF deficiency or dysfunction impairs primary hemostasis as well as the intrinsic pathway of secondary hemostasis. vWD is often asymptomatic, but mucocutaneous bleeding, GI bleeding, and menorrhagia may occur. Diagnosis is based on patient history and laboratory studies such as quantitative measurement of vWF and ristocetin cofactor assay. Treatment is indicated for symptomatic patients; prophylaxis is indicated for surgical candidates and involves desmopressin and concentrates containing vWF and factor VIII.

Epidemiological data refers to the US, unless otherwise specified.

Variants of von Willebrand disease [2]
Type Description Etiology Mechanism
Inherited von Willebrand disease [1]

Type 1 (80–85%)

  • VWD caused by mutations in the vWF gene
Type 2 (15–20%)
  • Dysfunctional vWF
Type 3 (∼ 3%)
Acquired von Willebrand disease (aVWD)
  • VWF deficiency that occurs secondary to other medical conditions [3]
  • Unknown

Deficiency or dysfunction of vWF leads to:

The severity of symptoms varies between the different types of vWD. Type 1 and avWD usually manifest more mildly; type 3 is the most severe form. [2][4]

History

  • Recurrent episodes of bleeding since childhood
  • Often positive family history

Laboratory studies [2]

Treatment is only indicated if symptoms occur or as prophylaxis before surgical procedures. [2]

Inherited von Willebrand disease

Acquired von Willebrand syndrome

Platelet aggregation inhibitors (e.g., aspirin, NSAIDs, clopidogrel) and intramuscular injections are contraindicated in von Willebrand disease because they further increase the risk of bleeding!

  1. What is von Willebrand Disease?. https://www.cdc.gov/ncbddd/vwd/facts.html. Updated: October 28, 2019. Accessed: August 4, 2020.
  2. The Diagnosis, Evaluation and Management of von Willebrand Disease. https://www.nhlbi.nih.gov/sites/default/files/media/docs/vwd.pdf. Updated: December 1, 2007. Accessed: August 4, 2020.
  3. Rick ME, Leung LLK, Tirnauer JS. Acquired von Willebrand Syndrome. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/acquired-von-willebrand-syndrome.Last updated: March 29, 2017. Accessed: May 30, 2018.
  4. Rick ME. Clinical presentation and diagnosis of von Willebrand disease. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/clinical-presentation-and-diagnosis-of-von-willebrand-disease?source=search_result&search=von%20willebrand-krankheit&selectedTitle=1~150.Last updated: May 6, 2016. Accessed: February 8, 2017.
  5. Committe Opinion: Von Willebrand Disease in Women. https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2013/12/von-willebrand-disease-in-women#. Updated: December 1, 2013. Accessed: August 10, 2020.