Inflammation-associated ADAMTS13 deficiency promotes formation of ultra-large von Willebrand factor
| Autor: | Clemens L. Bockmeyer, Ralf A. Claus, Ulrich Budde, Karim Kentouche, Reinhard Schneppenheim, Wolfgang Lösche, Konrad Reinhart, Frank M. Brunkhorst |
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| Jazyk: | angličtina |
| Rok vydání: | 2008 |
| Předmět: | |
| Zdroj: | Haematologica, Vol 93, Iss 1 (2008) |
| Druh dokumentu: | article |
| ISSN: | 0390-6078 1592-8721 |
| DOI: | 10.3324/haematol.11677 |
| Popis: | 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. |
| Databáze: | Directory of Open Access Journals |
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