Sun R, Li T, Ren W H
Department of Infectious Diseases, Shandong Provincial Hospital, Shandong University, Jinan 250021, China.
Zhonghua Gan Zang Bing Za Zhi. 2024 Mar 20;32(3):279-283. doi: 10.3760/cma.j.cn501113-20231129-00248.
Hepatic sinusoidal obstruction syndrome (HSOS) is a type of secondary vascular disease of the liver that is mainly associated with the ingestion of pyrrole alkaloids (PAs) and hematopoietic stem cell transplantation (HSCT) treatment, resulting in severe liver dysfunction, multiple organ failure, and even death. Hepatic sinusoidal dilatation and obstruction, hepatocyte coagulative necrosis, and hepatic lobular inflammation are the main pathological manifestations of HSOS. The key initiating process for the pathogenesis of HSOS is damage to liver sinusoidal endothelial cells (LSECs). Currently, it is believed that LSECs are damaged by the involvement of multiple etiologies and mechanisms, and secondary coagulation and fibrinolysis disorders, oxidative stress, and inflammatory responses are the occurrence contributors to HSOS; however, the mechanism has not been fully elucidated. Therefore, the role of immune-inflammatory mechanisms has received increasing attention in LSEC damage. This article provides an overview of the epidemiology, etiology, and pathological changes of HSOS and reviews the physiological functions, common etiological damage mechanisms, and the key role of LSEC damage in the pathogenesis of HSOS, with a special focus on the role and research progress of immune-inflammatory mechanisms for LSEC damage in recent years. Furthermore, we believe that in-depth study and elucidation of the role of immune-inflammatory mechanisms in LSEC damage and the pathogenesis of HSOS and diagnosis will provide feasible research and development ideas for the screening and identification of new markers and drug treatment targets for HSOS.
肝窦阻塞综合征(HSOS)是一种肝脏继发性血管疾病,主要与吡咯生物碱(PAs)摄入及造血干细胞移植(HSCT)治疗相关,可导致严重肝功能障碍、多器官功能衰竭甚至死亡。肝窦扩张与阻塞、肝细胞凝固性坏死及肝小叶炎症是HSOS的主要病理表现。HSOS发病机制的关键起始过程是肝窦内皮细胞(LSECs)损伤。目前认为,LSECs损伤涉及多种病因和机制,继发性凝血和纤溶紊乱、氧化应激及炎症反应是HSOS发生的促成因素;然而,其机制尚未完全阐明。因此,免疫炎症机制在LSECs损伤中的作用受到越来越多的关注。本文概述了HSOS的流行病学、病因及病理变化,综述了LSECs的生理功能、常见病因损伤机制以及LSECs损伤在HSOS发病机制中的关键作用,特别关注近年来免疫炎症机制对LSECs损伤的作用及研究进展。此外,我们认为深入研究并阐明免疫炎症机制在LSECs损伤及HSOS发病机制和诊断中的作用,将为HSOS新标志物的筛选鉴定及药物治疗靶点提供可行的研发思路。