【Annu Rev Med－编译】冯·威利布兰德病（血管性血友病）的新观念
发布于 2005-01-18 · 浏览 1002 · IP 四川四川
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冯·威利布兰德病（血管性血友病）的新观念
血管假性血友病因子（VWF）作为一种血管外的衔接分子，在血管损伤处使血小板和血管外基质结合。其结合在止血中起到了决定性的作用。缺乏血小板黏附导致的出血是典型的血管性血友病，然而太多的血小板黏附会导致血栓性血小板减少性紫癜。VWF或血小板糖蛋白Ib变异能够减少或增加血小板黏附的亲和力。荒谬的是，亲和力任一方向的改变都会引起出血。结晶学研究对所有这些表型从分子水平进行了阐释。对1型血管性血友病的临床研究对血浆VWF水平和出血或血栓形成的危险性之间的关系进行了说明。新的数
据显示，VWF水平是出血或血栓形成的危险因素的有效生物指标，可以为这些相反的病症的治疗和预防提供参考。
Annual Review of Medicine
Volume 56: Pages 173-191, 2005 (doi:10.1146/annurev.med.56.082103.104713)
First published online as a Review in Advance on Aug. 30, 2004
NEW CONCEPTS IN VON WILLEBRAND DISEASE
J. Evan Sadler-
Howard Hughes Medical Institute, Department of Medicine, Department of Biochemistry and Molecular Biophysics, Washington University School of Medicine, St. Louis, Missouri 63110; email: esadler@im.wustl.edu
Von Willebrand factor (VWF) behaves as an extracellular adapter molecule, linking platelets to the extracellular matrix at sites of vascular injury. These interactions are crucial for hemostasis. Too little platelet adhesion causes bleeding that is typical of von Willebrand disease, whereas too much platelet adhesion may cause thrombotic thrombocytopenic purpura. Mutations in VWF or platelet glycoprotein Ib can either reduce or increase the affinity of platelet binding. Paradoxically, affinity changes in either direction cause bleeding. Crystallographic studies now suggest molecular explanations for all of these phenotypes. Clinical investigations of von Willebrand disease type 1 are defining the relationship between plasma VWF level and the risk of bleeding or thrombosis. Emerging data suggest that VWF level is a useful biomarker for the risk of either bleeding or thrombosis and could be incorporated into a comprehensive approach to treat or prevent these adverse events.
血管假性血友病因子（VWF）作为一种血管外的衔接分子，在血管损伤处使血小板和血管外基质结合。其结合在止血中起到了决定性的作用。缺乏血小板黏附导致的出血是典型的血管性血友病，然而太多的血小板黏附会导致血栓性血小板减少性紫癜。VWF或血小板糖蛋白Ib变异能够减少或增加血小板黏附的亲和力。荒谬的是，亲和力任一方向的改变都会引起出血。结晶学研究对所有这些表型从分子水平进行了阐释。对1型血管性血友病的临床研究对血浆VWF水平和出血或血栓形成的危险性之间的关系进行了说明。新的数
据显示，VWF水平是出血或血栓形成的危险因素的有效生物指标，可以为这些相反的病症的治疗和预防提供参考。
Annual Review of Medicine
Volume 56: Pages 173-191, 2005 (doi:10.1146/annurev.med.56.082103.104713)
First published online as a Review in Advance on Aug. 30, 2004
NEW CONCEPTS IN VON WILLEBRAND DISEASE
J. Evan Sadler-
Howard Hughes Medical Institute, Department of Medicine, Department of Biochemistry and Molecular Biophysics, Washington University School of Medicine, St. Louis, Missouri 63110; email: esadler@im.wustl.edu
Von Willebrand factor (VWF) behaves as an extracellular adapter molecule, linking platelets to the extracellular matrix at sites of vascular injury. These interactions are crucial for hemostasis. Too little platelet adhesion causes bleeding that is typical of von Willebrand disease, whereas too much platelet adhesion may cause thrombotic thrombocytopenic purpura. Mutations in VWF or platelet glycoprotein Ib can either reduce or increase the affinity of platelet binding. Paradoxically, affinity changes in either direction cause bleeding. Crystallographic studies now suggest molecular explanations for all of these phenotypes. Clinical investigations of von Willebrand disease type 1 are defining the relationship between plasma VWF level and the risk of bleeding or thrombosis. Emerging data suggest that VWF level is a useful biomarker for the risk of either bleeding or thrombosis and could be incorporated into a comprehensive approach to treat or prevent these adverse events.
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