Hidden behind thromboinflammation: revealing the roles of von Willebrand factor in sickle cell disease pathophysiology.
Hidden behind thromboinflammation: revealing the roles of von Willebrand factor in sickle cell disease pathophysiology.
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DOI：
10.1097/moh.0000000000000755
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发表时间：
2023-05-01
影响因子：
3.2
通讯作者：
Lam, Wilbur A.
中科院分区：
文献类型：
--
作者：
Vital, Eudorah F.;Lam, Wilbur A.
这篇综述提供了SCD病理生理学的最新进展，特别关注血管性血友病因子- ADAMTS13轴失调的作用。在讨论最近的进展时，我们希望鼓励围绕SCD治疗靶点的新的和正在进行的讨论。在过去的五年中，VWF在SCD病理生理中的作用得到了强调。它目前是正在进行的临床试验研究的目标。SCD的病理生理是多方面的，因为它涉及全系统的血管激活、血液流变学改变、免疫激活和凝血作用。SCD中VWF的过量存在，特别是其最大的多聚体形式，极大地促进了其发病机制。了解SCD中大VWF多聚体存在的分子机制将有助于进一步了解SCD的发病机制，并为治疗提供特定的靶点。
This review provides an update on SCD pathophysiology with a particular focus on the role of the von Willebrand Factor – ADAMTS13 axis dysregulation. In discussing recent developments, we hope to encourage new and ongoing discussions surrounding therapeutic targets for SCD. Within the last five years, the role of VWF in the pathophysiology of SCD has been emphasized. It is currently a target of study in ongoing clinical trials. The pathophysiology of SCD is multifaceted, as it involves systemwide vascular activation, altered blood rheology, immune activation, and coagulative involvement. The presence of VWF in excess in SCD, particularly in its largest multimeric form, greatly contributes to its pathogenesis. Understanding the molecular mechanisms that underly the presence of large VWF multimers in SCD will provide further insight into the pathogenesis of SCD and provide specific targets for therapy.