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Homeostatic imbalance between von Willebrand factor and ADAMTS13 on hemorrhagic shock-induced endotheliopathy.

Created on 03 Sep 2026

Authors

Yi Wang, Sarah Abdullah, Feng Wu, Katie Houck, Roumen Vesselinov, Xiaoyun Fu, Lin Zou, Myung Park, Jing-Fei Dong, Rosemary Kozar

Published in

Haematologica. Sep 03, 2026. Epub Sep 03, 2026.

Abstract

Endothelial dysfunction in patients after severe injury with hemorrhagic shock is associated with excessive release of ultralarge von Willebrand factor (VWF) without a corresponding increase in ADAMTS13 (A disintegrin and metalloprotease thrombospondin type 1 motifs, member 13), resulting in acquired ADAMTS13 deficiency and VWF hyperadhesive activity. We hypothesized that the VWF to ADAMTS13 imbalance is exacerbated by increased VWF oxidation, rendering it resistant to cleavage by ADAMTS13. Plasma indices of endotheliopathy and coagulopathy in 100 hemorrhagic shock patients were obtained, ADAMTS13-to-VWF ratio was calculated and correlated with indices of adverse outcomes using Classification and Regression Tree modeling. A novel mass spectrometry method was used to quantify the rate of VWF cleavage in individual patients. In a mouse model of polytrauma and hemorrhagic shock, a single dose of human recombinant ADAMTS13 was administered and VWF cleavage, VWF activity, and parameters of lung dysfunction examined. We detected a reduced rate of cleavage and increased oxidation rate of selective methionine residues of VWF in these patients. In mice, recombinant ADAMTS13 decreased the adhesive activity and enhanced cleavage of plasma VWF, and improved parameters of lung dysfunction. The present study supports the conclusion that an imbalance between VWF and ADAMTS13 drives, at least in part, the endotheliopathy of trauma and the associated adverse outcomes and suggests a potential therapeutic target for severely injured patients with hemorrhagic shock.

PMID:
42687817
Bibliographic data and abstract were imported from PubMed on 03 Sep 2026.

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