肝窦阻塞综合征的研究进展

Research advances in hepatic sinusoidal obstruction syndrome

肝窦阻塞综合征(HSOS)也被称为肝小静脉闭塞病,是一类由各种原因导致的肝血窦阻塞和肝小静脉闭塞纤维化的肝脏血管性疾病。本文系统阐述了HSOS的认识过程和命名演变、病因和发病机制、临床表现、诊断与鉴别诊断、预防和治疗等方面的研究进展。HSOS可发生于接受骨髓造血干细胞移植清髓处理、放射/化学治疗和摄入含吡咯生物碱药物的人群,常见临床表现为腹胀、肝区胀痛、腹水、黄疸、肝大等。针对不同病因,HSOS的诊断标准也不相同,需要与其他药物性肝病、肝静脉流出道梗阻等疾病相鉴别。去纤苷和低分子肝素分别对造血干细胞和吡咯生物碱相关的HSOS具有治疗作用,奥沙利铂化疗导致的HSOS目前尚无有效治疗药物。

Hepatic sinusoidal obstruction syndrome(HSOS),also known as hepatic veno-occlusive disease,is hepatic vascular disease of hepatic sinusoidal obstruction and hepatic venular occlusion and fibrosis due to various causes.This article systematically elaborates on the research advances in HSOS from the aspects of understanding and naming,etiology and pathogenesis,clinical manifestation,diagnosis and differential diagnosis,prevention,and treatment.HSOS can occur in patients receiving bone marrow hematopoietic stem cell transplantation,radiotherapy/chemotherapy,and medication containing pyrrolidine alkaloids,and the common clinical manifestations of HSOS include abdominal distension,distending pain in the liver area,ascites,jaundice,and hepatomegaly.The diagnostic criteria for HSOS vary with etiology,and it needs to be differentiated from other diseases such as drug-induced liver diseases and hepatic venous outflow tract obstruction.Defibrotide and low-molecular-weight heparin have a therapeutic effect on HSOS associated with hematopoietic stem cells and pyrrolidine alkaloids,respectively,and there are currently no effective drugs for HSOS caused by oxaliplatin chemotherapy.