UDC

616-005.1

DOI

10.37482/2687-1491-Z232

Authors

Maria V. Kalashnikova* ORCID: https://orcid.org/0009-0003-2247-0425
Nushik S. Sarkisyan* ORCID: https://orcid.org/0000-0003-3512-5738
*Stavropol Plague Control Research Institute
(Stavropol, Russia)

Abstract

The review presents an analysis of literature data on the role of von Willebrand factor (VWF) in the system of haemostasis and inflammation as well as provides a pathophysiological assessment of the influence of VWF interaction with pathogenic microbiological agents on its metabolism and of the role of the metalloproteinase ADAMTS-13 in this process. The structure, functions and metabolism of VWF in pathological conditions are described. Data are presented indicating that the release of VWF from endothelial cells promotes the binding and decrease in the activity of ADAMTS-13, which regulates the functional activity of VWF. This, in turn, leads to the accumulation of ultralarge VWF multimers in the bloodstream, inducing the development of thrombosis. It is noted that biologically active substances involved in pathological processes act as stimulators of VWF exocytosis from Weibel–Palade bodies, which results in the development of microcirculatory disorders. The review aimed to assess the importance of VWF in the pathogenesis of inflammatory and thrombotic disorders. The sample consisted of original articles and short reports published from 2005 to 2022 and included in the PubMed, eLIBRARY.RU and Cyberleninka databases and addressing the pathophysiological role of VWF in maintaining systemic inflammation. The following search and selection strategy for scientific articles was applied using MeSH-indexed terms: von Willebrand factor [Supplementary Concept], ADAMTS-13 [MeSH Terms], systemic inflammation [MeSH Terms], and thrombotic disorders [MeSH Terms]. The analysis of scientific publications allows us to consider VWF as a marker of both haemostasis and inflammation. The influence of infectious agents on its metabolism is pointed out: during inflammation, bacteria associated with VWF multimers overcome the haemodynamic effects of the bloodstream, evade the immune cells and become fixed on the surface of the endothelium, causing endovascular disorders.

Keywords

von Willebrand factor, Weibel–Palade bodies, ADAMTS-13, immunothrombosis, systemic inflammation

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