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Semin Thromb Hemost 2008; 34(7): 663-669
DOI: 10.1055/s-0028-1104545
© Thieme Medical Publishers

Von Willebrand Factor: Another Janus-Faced Hemostasis Protein

Massimo Franchini1 , Pier Mannuccio Mannucci2
  • 1Immunohematology and Transfusion Centre, Department of Pathology and Laboratory Medicine, University Hospital of Parma, Italy
  • 2Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Medicine and Medical Specialties, University of Milan and IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation, Milan, Italy
Further Information

Publication History

Publication Date:
15 December 2008 (online)

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ABSTRACT

von Willebrand factor (VWF) is a multimeric plasma protein that mediates platelet adhesion and aggregation at sites of vascular injury and also acts as a protective carrier of factor VIII. Although the acquired or inherited deficiency of VWF is associated with a bleeding tendency, there is increasing evidence that VWF also plays a pivotal role in thrombosis. The presence in plasma of unusually large VWF multimers, due to the congenital or acquired deficiency of the VWF-cleaving metalloproteinase ADAMTS13, has been implicated in the pathogenesis of thrombotic thrombocytopenic purpura. In addition, high plasma levels of VWF have been associated with an increased risk of atherothrombosis. The current article reviews the literature and provides evidence for the two-faced character that VWF therefore represents.

KEYWORDS

von Willebrand factor - thrombosis - thrombotic thrombocytopenic purpura - coronary artery disease - stroke

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Massimo FranchiniM.D. 

Immunohematology and Transfusion Center, Department of Pathology and Laboratory Medicine

University Hospital of Parma, Italy

Email: massimo.franchini@azosp.vr.it