隐藏在血栓炎症背后:揭示血管性血友病因子在镰状细胞病病理生理中的作用。

IF 3.1 3区 医学 Q2 HEMATOLOGY Current Opinion in Hematology Pub Date : 2023-05-01 Epub Date: 2023-01-12 DOI:10.1097/MOH.0000000000000755
Eudorah F Vital, Wilbur A Lam
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引用次数: 1

摘要

综述目的:本综述提供了镰状细胞病(SCD)病理生理学的最新进展,特别关注血管性血友病因子(VWF) - ADAMTS13轴的失调,该轴有助于其发病。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。最近发现:近5年来,VWF在SCD病理生理中的作用得到了进一步的阐明,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、免疫反应和凝固途径的激活。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。
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Hidden behind thromboinflammation: revealing the roles of von Willebrand factor in sickle cell disease pathophysiology.

Purpose of review: This review provides an update on the pathophysiology of sickle cell disease (SCD) with a particular focus on the dysregulation of the von Willebrand factor (VWF) - ADAMTS13 axis that contributes to its pathogenesis. In discussing recent developments, we hope to encourage new and ongoing discussions surrounding therapeutic targets for SCD.

Recent findings: Within the last 5 years, the role of VWF in the pathophysiology of SCD has been further elucidated and is now a target of study in ongoing clinical trials.

Summary: The pathophysiology of SCD is multifaceted, as it involves systemwide vascular activation, altered blood rheology, and the activation of immune responses and coagulative pathways. 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.

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来源期刊
CiteScore
6.60
自引率
3.10%
发文量
78
审稿时长
6-12 weeks
期刊介绍: ​​​​​​​​Current Opinion in Hematology is an easy-to-digest bimonthly journal covering the most interesting and important advances in the field of hematology. Its hand-picked selection of editors ensure the highest quality selection of unbiased review articles on themes from nine key subject areas, including myeloid biology, Vascular biology, hematopoiesis and erythroid system and its diseases.
期刊最新文献
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