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

被引：108

作者：

Bockmeyer, Clemens L. ^{[1
]}

Claus, Ralf A. ^{[1
]}

Budde, Ulrich ^{[2
]}

Kentouche, Karim ^{[3
]}

Schneppenheim, Reinhard ^{[4
]}

Loesche, Wolfgang ^{[1
]}

Reinhart, Konrad ^{[1
]}

Brunkhorst, Frank M. ^{[1
]}

机构：

[1] Friedrich Schiller Univ, Univ Hosp, Dept Anaesthesiol & Intens Care Med, Jena, Germany

[2] Lab Assoc Prof Arndt & Partners, Coagulat Lab, Hamburg, Germany

[3] Friedrich Schiller Univ, Univ Hosp, Dept Pediat, Jena, Germany

[4] Childrens Univ Hosp Hamburg Eppendorf, Dept Pediat Hematol & Oncol, Hamburg, Germany

来源：

HAEMATOLOGICA | 2007年 / 93卷 / 01期

关键词：

ADAMTS13; extracorporeal circulation; sepsis; systemic inflammation; organ dysfunction; platelets; thrombotic microangiopathy;

D O I：

10.3324/haematol.11677

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：137 / 140

页数：4

共 14 条

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