New concepts in von Willebrand disease

Von Willebrand factor (VWF) behaves as an extracellular adapter molecule, linking platelets to the extracellular matrix at sites of vascular injury. These interactions are crucial for hemostasis. Too little platelet adhesion causes bleeding that is typical of von Willebrand disease, whereas too much...

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Bibliographic Details
Published in:Annual review of medicine 2005-01, Vol.56 (1), p.173-191
Main Author: Sadler, J Evan
Format: Article
Language:English
Subjects:
Blood clots
Disease
Genotype & phenotype
Glycoproteins
Hematology
Hemorrhage - genetics
Hemorrhage - physiopathology
Hemostasis - genetics
Hemostasis - physiology
Humans
Molecules
Mutation
Platelet Adhesiveness - genetics
Platelet Adhesiveness - physiology
Platelet Membrane Glycoproteins - genetics
Purpura, Thrombotic Thrombocytopenic - genetics
Purpura, Thrombotic Thrombocytopenic - physiopathology
Risk Factors
von Willebrand Diseases - classification
von Willebrand Diseases - diagnosis
von Willebrand Diseases - genetics
von Willebrand Diseases - physiopathology
von Willebrand Factor - genetics
von Willebrand Factor - physiology
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Summary:Von Willebrand factor (VWF) behaves as an extracellular adapter molecule, linking platelets to the extracellular matrix at sites of vascular injury. These interactions are crucial for hemostasis. Too little platelet adhesion causes bleeding that is typical of von Willebrand disease, whereas too much platelet adhesion may cause thrombotic thrombocytopenic purpura. Mutations in VWF or platelet glycoprotein Ib can either reduce or increase the affinity of platelet binding. Paradoxically, affinity changes in either direction cause bleeding. Crystallographic studies now suggest molecular explanations for all of these phenotypes. Clinical investigations of von Willebrand disease type 1 are defining the relationship between plasma VWF level and the risk of bleeding or thrombosis. Emerging data suggest that VWF level is a useful biomarker for the risk of either bleeding or thrombosis and could be incorporated into a comprehensive approach to treat or prevent these adverse events.
ISSN:0066-4219
1545-326X
DOI:10.1146/annurev.med.56.082103.104713