Von Willebrand factor and ADAMTS13 in arterial thrombosis: a systematic review and meta-analysis.
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.
Department of Hematology, Erasmus University Medical Center, Rotterdam, The Netherlands.
Michelle A H
MA
Sonneveld
Department of Hematology, Erasmus University Medical Center, Rotterdam, The Netherlands.
Moniek P M
MP
de Maat
Department of Hematology, Erasmus University Medical Center, Rotterdam, The Netherlands. Electronic address: f.leebeek@erasmusmc.nl.
Frank W G
FW
Leebeek
D051722:ADAM Proteins / Q000235:genetics / Q000378:metabolism*; D000071120:ADAMTS13 Protein; D000818:Animals; D001158:Arteries / Q000378:metabolism* / Q000473:pathology; D002318:Cardiovascular Diseases / Q000209:etiology; D004195:Disease Models, Animal; D006801:Humans; D051379:Mice; D013927:Thrombosis / Q000235:genetics / Q000378:metabolism*; D014842:von Willebrand Diseases / Q000150:complications / Q000235:genetics / Q000378:metabolism; D014841:von Willebrand Factor / Q000235:genetics / Q000378:metabolism*
D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
ADAMTS13; Coronary heart disease; Ischemic stroke; Myocardial infarction; von Willebrand factor