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造血干细胞移植后肝静脉闭塞病

Hepatic veno-occlusive disease following hematopoietic stem cell transplantation.

作者信息

Richardson P, Guinan E

机构信息

Department of Adult Oncology, Dana-Farber Cancer Institute, 44 Binney Street, Boston, MA 02115, USA.

出版信息

Acta Haematol. 2001;106(1-2):57-68. doi: 10.1159/000046590.

DOI:10.1159/000046590
PMID:11549778
Abstract

The clinical syndrome of hepatic veno-occlusive disease (VOD) is one of the most common and serious complications following hematopoietic stem cell transplantation (SCT). High-dose chemotherapy or chemoradiation therapy in the context of autologous and allogeneic SCT can profoundly injure sinusoidal endothelium and hepatocytes within zone 3 of the liver acinus, producing the clinical syndrome of hepatomegaly and/or right upper quadrant pain with jaundice and fluid retention, typically manifest as weight gain. The incidence is variable and ranges from 10 to 60%. Mild to moderate disease is characterized by eventual complete resolution. In contrast, severe disease frequently results in multiorgan failure and death. The purpose of this review is to discuss the pathophysiology and clinical features of VOD, and the current status and future directions of research for both prevention and treatment.

摘要

肝静脉闭塞病(VOD)的临床综合征是造血干细胞移植(SCT)后最常见且严重的并发症之一。在自体和异基因SCT中,大剂量化疗或放化疗可严重损伤肝腺泡3区的窦状内皮细胞和肝细胞,产生肝肿大和/或右上腹疼痛伴黄疸及液体潴留的临床综合征,典型表现为体重增加。其发病率不一,范围在10%至60%之间。轻度至中度疾病的特点是最终可完全缓解。相比之下,严重疾病常导致多器官功能衰竭和死亡。本综述的目的是讨论VOD的病理生理学和临床特征,以及预防和治疗的研究现状与未来方向。

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