The Pathophysiology of Von Willebrand Disease

In: Science

Submitted By crimson87
Words 671
Pages 3
The congenital bleeding disorder, von Willebrand Disease, is the most frequently occurring congenital hemorrhagic disorder. It affects about one percent of the population and occurs in both sexes, as it is passed down genetically via autosomal dominant inheritance. The main presenting symptom of the disease is abnormal bleeding of mucosal membranes, usually occurring after a challenge to the body’s hemostatic ability such as surgery or a dental extraction. Menorrhagia in women can also be indicative of von Willebrand Disease. This abnormal mucosal bleeding is attributed to a quantitative deficiency or structural abnormality in the body’s supply of von Willebrand Factor, which is necessary for the body’s hemostasis and coagulative function. Within the human circulatory system, von Willebrand Factor, or VWF, has three main functions. Its primary function is to mediate platelet adhesion to the subendothelial collagen of blood vessel walls when they become damaged. This occurs most frequently in capillaries and arterioles, where the flow rate of blood is highest and desquamation of the endothelial lining is the most common. VWF is also the carrier molecule for the plasma procoagulant, Factor VIII. They form the VWF/VIII complex, which prevents the proteolysis of Factor VIII and prolongs its plasma half-life from only a few moments to eight to twelve hours. von Willebrand Factor is also responsible for mediating platelet-to-platelet aggregation through the release of glycoprotein stimulants. von Willebrand Disease is the product of either structural or quantitative dysfunction of von Willebrand Factor, which impairs primary hemostasis by reducing platelet adhesion. This is what leads to the mucocutaneous hemorrhaging. If the deficiency of VWF is extreme, it causes a deficiency of Factor VIII, which can lead to symptoms of Hemophilia A. The vast majority of…...

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