Complement involvement in sickle cell disease

IF 3.2 3区医学 Q1 MEDICINE, GENERAL & INTERNAL

Presse Medicale Pub Date : 2023-11-14 DOI:10.1016/j.lpm.2023.104205

Marie-Sophie Meuleman , Lubka T. Roumenina , Anne Grunenwald

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Abstract

Sickle Cell Disease (SCD) is a hereditary blood disorder characterized by the presence of abnormal hemoglobin, leading to the formation of sickle-shaped red blood cells, causing vaso-occlusion. Inflammation is a key component of the pathophysiology of SCD, contributing to the vascular complications and tissue damage. This review is centered on exploring the role of the inflammatory complement system in the pathophysiology of SCD. Our goal is to offer a comprehensive summary of the existing evidence regarding complement activation in patients with SCD, encompassing both steady-state conditions and episodes of vaso-occlusive events. Additionally, we will discuss the proposed mechanisms by which the complement system may contribute to tissue injury in this pathology. Finally, we will provide an overview of the available evidence concerning the effectiveness of therapeutic interventions aimed at blocking the complement system in the context of SCD and discuss the perspective of complement inhibition.

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补体参与镰状细胞病

镰状细胞病(SCD)是一种遗传性血液疾病，其特征是存在异常血红蛋白，导致镰状红细胞形成，引起血管闭塞。炎症是SCD病理生理的重要组成部分，可导致血管并发症和组织损伤。本文就炎性补体系统在SCD病理生理中的作用进行综述。我们的目标是提供一个关于SCD患者补体激活的现有证据的综合总结，包括稳态条件和血管闭塞事件的发作。此外，我们将讨论补体系统在这种病理中可能导致组织损伤的机制。最后，我们将概述有关SCD背景下旨在阻断补体系统的治疗干预有效性的现有证据，并讨论补体抑制的观点。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Presse Medicale 医学-医学：内科

自引率

3.70%

发文量

审稿时长

43 days

期刊介绍： Seule revue médicale "généraliste" de haut niveau, La Presse Médicale est l''équivalent francophone des grandes revues anglosaxonnes de publication et de formation continue. A raison d''un numéro par mois, La Presse Médicale vous offre une double approche éditoriale : - des publications originales (articles originaux, revues systématiques, cas cliniques) soumises à double expertise, portant sur les avancées médicales les plus récentes ; - une partie orientée vers la FMC, vous propose une mise à jour permanente et de haut niveau de vos connaissances, sous forme de dossiers thématiques et de mises au point dans les principales spécialités médicales, pour vous aider à optimiser votre formation.