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Von Willebrand disease (VWD) is the most common hereditary blood-clotting disorder in humans. An acquired form can sometimes result from other medical conditions. [ 1 ] It arises from a deficiency in the quality or quantity of von Willebrand factor (VWF), a multimeric protein that is required for platelet adhesion .
Von Willebrand factor (VWF) (German: [fɔn ˈvɪləbʁant]) is a blood glycoprotein that promotes hemostasis, specifically, platelet adhesion. It is deficient and/or defective in von Willebrand disease and is involved in many other diseases, including thrombotic thrombocytopenic purpura, Heyde's syndrome, and possibly hemolytic–uremic ...
Type III von Willebrand Disease is a severe bleeding disorder, like severe hemophilia type A or B. VWF acts in primary hemostasis to recruit platelets at a site of injury, and is also important in secondary hemostasis, acting as a chaperone for coagulation factor VIII (FVIII). [20]
The National Bleeding Disorders Foundation (NBDF) is a United States patient advocacy organization for the care and treatment of inheritable blood and bleeding disorders such as hemophilia and von Willebrand disease. Founded in 1948, NBDF, then known as the National Hemophilia Foundation, helps secure funding for treatment centers and develops ...
It is similar to the ristocetin cofactor assay but has the added benefit in that it helps in the diagnosis of type 2B/pseudo von Willebrand disease (vWD) and Bernard–Soulier syndrome because it uses patient's live endogenous platelets, whereas ristocetin cofactor assay tests the function of only the vWF and not the platelets. Ristocetin ...
This bleeding disorder is called "Von Willebrand Disease". The term "haemophilia" is derived from the term "haemorrhaphilia" which was used in a description of the condition written by Friedrich Hopff in 1828, while he was a student at the University of Zurich.
The important role of depletion of von Willebrand factor in aortic stenosis was first proposed in 1992 by Warkentin et al. [7] They noted a known association between aortic stenosis (in addition to other cardiac diseases) and acquired von Willebrand's disease type IIA, [20] which is corrected by surgical replacement of the aortic valve. They ...
Vonicog alfa, sold under the brand names Vonvendi and Veyvondi, is a medication used to control bleeding in adults with von Willebrand disease (an inherited bleeding disorder). [6] [5] [7] [8] It is a recombinant von Willebrand factor. [6] [5] The most common adverse reactions are generalized itching, vomiting, nausea, dizziness, and vertigo. [6]