Von Willebrand factor
Citation, DOI, disclosures and article data
At the time the article was created Craig Hacking had the following disclosures:
- Philips Australia, Paid speaker at Philips Spectral CT events (ongoing)
These were assessed during peer review and were determined to not be relevant to the changes that were made.View Craig Hacking's current disclosures
At the time the article was last revised Jeremy Jones had the following disclosures:
- Radiopaedia Events Pty Ltd, Jeremy is a director of Radiopedia Events Pty Ltd (ongoing)
These were assessed during peer review and were determined to not be relevant to the changes that were made.View Jeremy Jones's current disclosures
Von Willebrand factor (vWF) is a large hemostatic glycoprotein which serves several roles in platelet aggregation and the clotting cascade.
vWF is synthesized by vascular endothelial cells and megakaryocytes. A series of complex post-translational modifications result in the creation of physiologically active large-molecular-weight vWF multimers (LMWMs) and ultra large-molecular-weight multimers (ULMWMs) 1. It is encoded by the large VWF gene on the short arm of chromosome 12 3.
The active forms of vWF are released by injured endothelium, which promotes hemostasis by three mechanisms 2,3:
platelet adhesion: vWF binds to exposed collagen, which allows binding of GpIb resulting in platelet adhesion
platelet aggregation: vWF on adhered activated platelets irreversibly binds to GpIIb/IIIa to form the initial platelet plug
coagulation: vWF binds and stabilizes factor VIII in the clotting cascade, prolonging its half-life
Ongoing research is identifying further interactions of vWF in inflammation, hemorrhagic shock and traumatic brain injury 3.
Von Willebrand disease is a bleeding diathesis due to a deficiency in vWF. It is the most common inherited disorder of hemostasis.
History and etymology
It is named after Erik Adolf von Willebrand (1870-1949), Finnish physician and medical professor, who first described von Williebrand disease in 1926 2.
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- 2. Swami A & Kaur V. Von Willebrand Disease: A Concise Review and Update for the Practicing Physician. Clin Appl Thromb Hemost. 2017;23(8):900-10. doi:10.1177/1076029616675969 - Pubmed
- 3. Zeineddin A, Dong J, Wu F, Terse P, Kozar R. Role of Von Willebrand Factor After Injury: It May Do More Than We Think. Shock. 2021;55(6):717-22. doi:10.1097/SHK.0000000000001690 - Pubmed
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