The role of von Willebrand factor in the development of systemic inflammation, coagulopathy and organ dysfunctions: 616.151.511:616-002.16

Abstract

Summary. Von Willebrand factor (vWF) is a multimeric glycoprotein that plays a key role in platelet and plasma hemostasis. In recent years, the involvement of vWF in angiogenesis, apoptosis of normal and tumor cells has been revealed, and its transport role has been determined. The complex of vWF and enzyme ADAMTS-13, which is responsible for its splitting, is essential in the development of inflammatory processes. The mechanisms of vWF participation in inflammatory processes varies and include inter- action with microorganisms, blood cells and their degradation products, neutrophil extracellular traps, the complement system, etc. The unique qualities of the vWF contribute to the development and progression of coagulopathy in sepsis. Systemic inflam- mation enhances local inhibition of vWF cleavage and exacerbates an imbalance in the vWF/ADAMTS-13 ratio, which contributes to the development of intravascular coagulation. The formation of ultralarge vWF (ULvWF) multimers and ADAMTS-13 deficiency were detected in sepsis, severe COVID-19, craniocerebral and concomitant trauma, acute kidney injury, etc. The pathogenesis of the resulting multiple organ failure due to the accumulation of ULvWF is based on the development of microangiopathy and micro- vascular thrombosis. Possible methods for correcting disorders in the vWF/АDAMTS-13 system are the use of therapeutic plasma exchange, heparin, N-acetylcysteine, recombinant ADAMTS-13, and magnesium preparations.

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