Both neutrophil extracellular traps (NETs) and von Willebrand factor (VWF) are essential for thrombosis and inflammation. During these processes, a complex series of events, including endothelial activation, NET formation, VWF secretion, and blood cell adhesion, aggregation and activation, occurs in an ordered manner in the vasculature. The adhesive activity of VWF multimers is regulated by a specific metalloprotease ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motifs, member 13). Increasing evidence indicates that the interaction between NETs and VWF contributes to arterial and venous thrombosis as well as inflammation. Furthermore, contents released from activated neutrophils or NETs induce the reduction of ADAMTS13 activity, which may occur in both thrombotic microangiopathies (TMAs) and acute ischemic stroke (AIS). Recently, NET is considered as a driver of endothelial damage and immunothrombosis in COVID-19. In addition, the levels of VWF and ADAMTS13 can predict the mortality of COVID-19. In this review, we summarize the biological characteristics and interactions of NETs, VWF, and ADAMTS13, and discuss their roles in TMAs, AIS, and COVID-19. Targeting the NET-VWF axis may be a novel therapeutic strategy for inflammation-associated TMAs, AIS, and COVID-19.

中性粒细胞胞外捕获物（NETs）和血管性血友病因子（VWF）对于血栓形成和炎症至关重要。在这些过程中，一系列复杂的事件（包括内皮激活，NET形成，VWF分泌以及血细胞粘附，聚集和激活）在脉管系统中以有序的方式发生。VWF多聚体的粘附活性受特定金属蛋白酶ADAMTS13（具有血小板反应蛋白1型基序的双整合素和金属蛋白酶，成员13）调节。越来越多的证据表明，NET和VWF之间的相互作用会导致动脉和静脉血栓形成以及炎症。此外，从活化的中性粒细胞或NETs释放的内容物会导致ADAMTS13活性降低，这可能在血栓性微血管病（TMA）和急性缺血性卒中（AIS）中均会发生。最近，NET被认为是COVID-19中内皮损伤和免疫血栓形成的驱动因素。此外，VWF和ADAMTS13的水平可以预测COVID-19的死亡率。在这篇综述中，我们总结了NETs，VWF和ADAMTS13的生物学特性和相互作用，并讨论了它们在TMA，AIS和COVID-19中的作用。靶向NET-VWF轴可能是与炎症相关的TMA，AIS和COVID-19的新型治疗策略。