Haematologica, Vol 93, Issue 1, 137-140 doi:10.3324/haematol.11677
Copyright © 2008 by Ferrata Storti Foundation
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Hemostasis

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

Clemens L. Bockmeyer1, Ralf A. Claus1,, Ulrich Budde2, Karim Kentouche3, Reinhard Schneppenheim4, Wolfgang Lösche1, Konrad Reinhart1, Frank M. Brunkhorst1

1 Department of Anaesthesiology and Intensive Care Medicine, University Hospital, Friedrich-Schiller-University, Jena
2 Lab-Association Prof. Arndt and Partners, Coagulation Laboratory, Hamburg
3 Department of Pediatrics, University Hospital, Friedrich-Schiller-University, Jena and
4 Children's University Hospital Hamburg-Eppendorf, Department of Pediatric Hematology and Oncology, Germany

Correspondence: Ralf A. Claus, PhD, Dept. of Anaesthesiology and Intensive Care Medicine, Friedrich-Schiller-University, Erlanger Allee 101, D-07747 Jena, Germany. E-mail: ralf.claus{at}med.uni-jena.de

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.

Key words: ADAMTS13, extracorporeal circulation, sepsis, systemic inflammation, organ dysfunction, platelets, thrombotic microangiopathy.




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