Semin Thromb Hemost
DOI: 10.1055/s-0037-1605564
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

von Willebrand Factor and Venous Thromboembolism: Pathogenic Link and Therapeutic Implications

Paolo Calabrò1, *, Felice Gragnano1, *, Enrica Golia1, Erik Lerkevang Grove2, 3
  • 1Division of Cardiology, Department of Cardiothoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli,” Naples, Italy
  • 2Department of Cardiology, Aarhus University Hospital, Aarhus University, Aarhus, Denmark
  • 3Department of Health, Institute of Clinical Medicine, Aarhus University, Aarhus, Denmark
Further Information

Publication History

Publication Date:
12 September 2017 (eFirst)


Venous thromboembolism (VTE) is a frequent cause of disability and mortality worldwide. Von Willebrand factor (VWF) is a major determinant of hemostasis and clot formation, in both arteries and veins. Although VWF is mainly known for its role in arterial thrombosis, several studies suggest a pathogenic role for VWF and its regulator ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) in venous thrombosis. Nongenetic and genetic factors, including gene mutations and polymorphisms, aging, hormone status, ABO blood groups, and systemic inflammation, have been involved in the modulation of both VTE predisposition and plasma levels of VWF. In several clinical settings, including inflammatory disease and cancer, VWF and ADAMTS-13 are currently investigated as possible determinants of vein thrombosis. These data indicate VWF as a potential therapeutic target in the management of VTE. Several studies report unselective antagonism of VWF for drugs used in daily clinical practice, including heparin and statins. Selective inhibition of VWF pathway has recently been tested in animal models of arterial and venous thrombosis as a novel therapeutic strategy to prevent platelet aggregation and thrombosis, promote vein lumen recanalization, and improve vein valve competency with excellent safety profile. In this review, we summarize the role of VWF in VTE, focusing on clinical and potential therapeutic implications.

Authors' Contributions

All authors contributed to the conception and design of the article; drafting the article or revising it critically for important intellectual content; and final approval of the version to be published.

* The authors Paolo Calabrò and Felice Gragnano contributed equally to the manuscript.