Von Willebrand disease overview: Difference between revisions
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Von Willebrand disease (vWD) is the most common genetic coagulation disorder described in humans. It affects up to 1% of the population, although most cases are mild. Symptomatic vWD is much rare, ~1 in 10000. Von Willebrand disease arises from a qualitative or quantitative deficiency of [[von Willebrand factor]] ([[vWF]]), a large glycoprotein protein that is required for [[platelet]]<nowiki/>s to bind to collagen. [[vWF]] is therefore important in primary hemostasis. When the disease comes to medical attention, it usually presents in the typical manner for platelet disorders: mucosal bleeding and easy bruising. The disease is usually inherited in an autosomal dominant manner, although there are recessive forms as well, and it can also be acquired secondary to another disease. <ref>Ng et al. Diagnostic Approach to von Willebrand Disease. Blood 2015; 125(13): 2029-2037.</ref><ref>Blomback et al. Von Willebrand Disease Biology Hemophilia 2012; 18: 141-147.</ref><ref>Favarolo et al. Von Willebrand Disease and Platelet Disorders. Hemophilia 2014; 20: 59-64.</ref> | Von Willebrand disease (vWD) is the most common genetic coagulation disorder described in humans. It affects up to 1% of the population, although most cases are mild. Symptomatic [[vWD]] is much rare, ~1 in 10000. Von Willebrand disease arises from a qualitative or quantitative deficiency of [[von Willebrand factor]] ([[vWF]]), a large glycoprotein protein that is required for [[platelet]]<nowiki/>s to bind to collagen. [[vWF]] is therefore important in primary hemostasis. When the disease comes to medical attention, it usually presents in the typical manner for platelet disorders: mucosal bleeding and easy bruising. The disease is usually inherited in an autosomal dominant manner, although there are recessive forms as well, and it can also be acquired secondary to another disease. <ref>Ng et al. Diagnostic Approach to von Willebrand Disease. Blood 2015; 125(13): 2029-2037.</ref><ref>Blomback et al. Von Willebrand Disease Biology Hemophilia 2012; 18: 141-147.</ref><ref>Favarolo et al. Von Willebrand Disease and Platelet Disorders. Hemophilia 2014; 20: 59-64.</ref> | ||
==References== | ==References== | ||
Revision as of 15:41, 28 November 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Von Willebrand’s disease is an genetic coagulation disorder with resultant abnormality in platelet adhesion and aggregation.
Von Willebrand disease (vWD) is the most common genetic coagulation disorder described in humans. It affects up to 1% of the population, although most cases are mild. Symptomatic vWD is much rare, ~1 in 10000. Von Willebrand disease arises from a qualitative or quantitative deficiency of von Willebrand factor (vWF), a large glycoprotein protein that is required for platelets to bind to collagen. vWF is therefore important in primary hemostasis. When the disease comes to medical attention, it usually presents in the typical manner for platelet disorders: mucosal bleeding and easy bruising. The disease is usually inherited in an autosomal dominant manner, although there are recessive forms as well, and it can also be acquired secondary to another disease. [1][2][3]