炎症相关的ADAMTS13缺乏促进超大血管性血友病因子的形成。

Inflammation-associated ADAMTS13 deficiency promotes formation of ultra-large von Willebrand factor.

作者信息

Bockmeyer Clemens L, Claus Ralf A, Budde Ulrich, Kentouche Karim, Schneppenheim Reinhard, Lösche Wolfgang, Reinhart Konrad, Brunkhorst Frank M

机构信息

Department of Anaesthesiology and Intensive Care Medicine, University Hospital, Friedrich-Schiller-University, Erlanger Allee 101, D-07747 Jena, Germany.

出版信息

Haematologica. 2008 Jan;93(1):137-40. doi: 10.3324/haematol.11677.

DOI:10.3324/haematol.11677

PMID:18166799

Abstract

In a prospective, longitudinal study, we investigated the association between decreased ADAMTS13 activity and impaired hemostasis, as well as organ dysfunctions in patients with systemic inflammation due to extracorporeal cardiopulmonary circuit or with severe sepsis. Similar to negative acute phase proteins, ADAMTS13 activity declined stepwise according to the extent of inflammatory responses. A marked imbalance between ADAMTS13 activity and VWF antigen level was associated with the appearance of ultra-large VWF multimers in plasma, with organ dysfunction and lethality. Our data support the view that systemic inflammation results in an ADAMTS13 deficiency which activates hemostasis.

摘要

在一项前瞻性纵向研究中，我们调查了因体外心肺循环或严重脓毒症导致全身炎症的患者中，ADAMTS13活性降低与止血功能受损以及器官功能障碍之间的关联。与负急性期蛋白相似，ADAMTS13活性根据炎症反应的程度逐步下降。ADAMTS13活性与VWF抗原水平之间的明显失衡与血浆中超大型VWF多聚体的出现、器官功能障碍和致死率相关。我们的数据支持这样一种观点，即全身炎症会导致ADAMTS13缺乏，从而激活止血功能。