Von Willebrand factor and ADAMTS13 in arterial thrombosis: a systematic review and meta-analysis

Abstract Von Willebrand Factor (VWF) plays an important role in hemostasis by mediating platelet adhesion and aggregation. Ultralarge VWF multimers are cleaved by ADAMTS13 in smaller, less procoagulant forms. An association between high VWF levels and cardiovascular disease has frequently been repor...

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Bibliographic Details
Published in:Blood reviews 2014-07, Vol.28 (4), p.167-178
Main Authors: Sonneveld, Michelle A.H, de Maat, Moniek P.M, Leebeek, Frank W.G
Format: Article
Language:English
Subjects:
ADAM Proteins - genetics
ADAM Proteins - metabolism
ADAMTS13
ADAMTS13 Protein
Animals
Arteries - metabolism
Arteries - pathology
Cardiovascular Diseases - etiology
Coronary heart disease
Disease Models, Animal
Hematology, Oncology and Palliative Medicine
Humans
Ischemic stroke
Mice
Myocardial infarction
Thrombosis - genetics
Thrombosis - metabolism
von Willebrand Diseases - complications
von Willebrand Diseases - genetics
von Willebrand Diseases - metabolism
von Willebrand factor
von Willebrand Factor - genetics
von Willebrand Factor - metabolism
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Summary:Abstract Von Willebrand Factor (VWF) plays an important role in hemostasis by mediating platelet adhesion and aggregation. Ultralarge VWF multimers are cleaved by ADAMTS13 in smaller, less procoagulant forms. An association between high VWF levels and cardiovascular disease has frequently been reported, and more recently also an association has been observed between low ADAMTS13 levels and arterial thrombosis. We reviewed the current literature and performed meta-analyses on the relationship between both VWF and ADAMTS13 with arterial thrombosis. Most studies showed an association between high VWF levels and arterial thrombosis. It remains unclear whether ADAMTS13 is a causal independent risk factor because the association between low ADAMTS13 and arterial thrombosis is so far only shown in case-control studies. Prospective studies are awaited. A causal role for ADAMTS13 is supported by mice studies of cerebral infarction where the infusion of recombinant human ADAMTS13 reduced the infarct size.
ISSN:0268-960X
1532-1681
DOI:10.1016/j.blre.2014.04.003