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Abstract
von Willebrand factor (VWF) is a multimeric plasma protein that mediates platelet adhesion as well as platelet aggregation at sites of vascular injury and acts as a carrier of factor VIII. Although acquired or inherited VWF deficiency is associated with a bleeding tendency, there is increasing evidence that VWF has a pivotal role in thrombogenesis. In fact, while the presence in the plasma of unusually large VWF multimers, due to a congenital or acquired deficiency of a VWF-cleaving metalloprotease, has been implicated in the pathogenesis of thrombotic thrombocytopenic purpura, high plasma levels of VWF have been associated with an increased risk of both arterial and venous thrombosis. The role of VWF in normal and pathological hemostasis is discussed in this review, and important advances in the pathophysiology, diagnosis, and treatment of VWF-associated disorders are also described.
| Abbreviations | ||
| ADAMTS-13 | = | a disintegrin and metalloprotease, with thrombospondin-1-like domains |
| aVWS | = | acquired von Willebrand syndrome |
| CT | = | closure time |
| DDAVP | = | desmopressin |
| FVIII | = | factor VIII |
| FVIII:C | = | factor VIII coagulant |
| GP | = | glycoprotein |
| GPIIb-IIIa | = | integrin IIb 3 |
| HES | = | hydroxyethyl starch |
| HMWM | = | high-molecular-weight multimers of VWF |
| ISTH | = | International Society of Thrombosis and Haemostasis |
| MGUS | = | monoclonal gammopathy of undetermined significance |
| PFA-100 | = | platelet function analyzer (Dade-Behring, Marburg, Germany. This is the only commercially available platelet function analyzer) |
| RIPA | = | ristocetin-induced platelet agglutination |
| TTP | = | thrombotic thrombocytopenic purpura |
| VWD | = | von Willebrand disease |
| VWF | = | von Willebrand factor |
| VWF:Ag | = | von Willebrand factor antigen |
| VWF:CB | = | von Willebrand factor collagen binding |
| VWF:RCo | = | von Willebrand factor ristocetin cofactor |
| Abbreviations | ||
| ADAMTS-13 | = | a disintegrin and metalloprotease, with thrombospondin-1-like domains |
| aVWS | = | acquired von Willebrand syndrome |
| CT | = | closure time |
| DDAVP | = | desmopressin |
| FVIII | = | factor VIII |
| FVIII:C | = | factor VIII coagulant |
| GP | = | glycoprotein |
| GPIIb-IIIa | = | integrin IIb 3 |
| HES | = | hydroxyethyl starch |
| HMWM | = | high-molecular-weight multimers of VWF |
| ISTH | = | International Society of Thrombosis and Haemostasis |
| MGUS | = | monoclonal gammopathy of undetermined significance |
| PFA-100 | = | platelet function analyzer (Dade-Behring, Marburg, Germany. This is the only commercially available platelet function analyzer) |
| RIPA | = | ristocetin-induced platelet agglutination |
| TTP | = | thrombotic thrombocytopenic purpura |
| VWD | = | von Willebrand disease |
| VWF | = | von Willebrand factor |
| VWF:Ag | = | von Willebrand factor antigen |
| VWF:CB | = | von Willebrand factor collagen binding |
| VWF:RCo | = | von Willebrand factor ristocetin cofactor |