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Nov 7, 2023
Von Willebrand factor (VWF) is a protein in the blood that plays a crucial role in blood clotting. It is named after Erik Adolf von Willebrand, a Finnish physician who first described the condition associated with its deficiency.
VWF is produced by cells in the blood vessel walls and megakaryocytes (large bone marrow cells). It circulates in the blood as a large multimeric protein, which means it consists of multiple subunits linked together. VWF has two main functions:
1. Platelet adhesion: VWF binds to platelets and helps them adhere to the damaged blood vessel walls, forming a plug to stop bleeding.
2. Carrying factor VIII: VWF acts as a carrier protein for factor VIII, another important clotting protein. It protects factor VIII from degradation and helps transport it to the site of injury, where it is needed for blood clot formation.
Deficiency or dysfunction of von Willebrand factor can lead to a bleeding disorder called von Willebrand disease (VWD). This condition is characterized by prolonged bleeding time, easy bruising, and excessive bleeding after injury or surgery. VWD is the most common inherited bleeding disorder, and it can be inherited in an autosomal dominant or recessive manner.
Treatment for von Willebrand disease typically involves replacing the deficient or dysfunctional VWF with VWF concentrates or desmopressin, a medication that stimulates the release of VWF from storage sites in the body.
VWF is produced by cells in the blood vessel walls and megakaryocytes (large bone marrow cells). It circulates in the blood as a large multimeric protein, which means it consists of multiple subunits linked together. VWF has two main functions:
1. Platelet adhesion: VWF binds to platelets and helps them adhere to the damaged blood vessel walls, forming a plug to stop bleeding.
2. Carrying factor VIII: VWF acts as a carrier protein for factor VIII, another important clotting protein. It protects factor VIII from degradation and helps transport it to the site of injury, where it is needed for blood clot formation.
Deficiency or dysfunction of von Willebrand factor can lead to a bleeding disorder called von Willebrand disease (VWD). This condition is characterized by prolonged bleeding time, easy bruising, and excessive bleeding after injury or surgery. VWD is the most common inherited bleeding disorder, and it can be inherited in an autosomal dominant or recessive manner.
Treatment for von Willebrand disease typically involves replacing the deficient or dysfunctional VWF with VWF concentrates or desmopressin, a medication that stimulates the release of VWF from storage sites in the body.
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