期刊
HAEMATOLOGICA
卷 93, 期 1, 页码 137-140出版社
FERRATA STORTI FOUNDATION
DOI: 10.3324/haematol.11677
关键词
ADAMTS13; extracorporeal circulation; sepsis; systemic inflammation; organ dysfunction; platelets; thrombotic microangiopathy
类别
资金
- Thuringian Ministry of Science and Arts (TMWFK) [B-309-00014]
- Centre for Clinical Research, Jena (IZKF)
- 'Forderverein' Friedrich-Schiller University, Jena
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.
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