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造血干细胞移植后肝窦阻塞综合征的治疗和预防

Author
Robert S Negrin, MD
Section Editor
Nelson J Chao, MD
Deputy Editor
Alan G Rosmarin, MD
Translators
张红宾, 副主任医师，副教授

引言

肝窦阻塞综合征(sinusoidal obstruction syndrome, SOS)，也曾被称作肝小静脉闭塞病(veno-occlusive disease, VOD)，是异基因和自体造血干细胞移植(hematopoietic cell transplantation, HCT)中最可怕的并发症之一，引起相当一部分的移植相关死亡，重度SOS几乎百分百致死。

SOS以肝肿大、右上腹痛、黄疸和腹水为特征，最常发生于接受HCT的患者，少部分发生于使用化疗药物的非移植患者，以及生物碱毒素摄入、进行大剂量放疗后或者肝移植的患者。SOS的临床表现类似于巴德-吉亚利综合征；然而，SOS中的肝静脉流出梗阻是由终末肝小静脉和肝窦闭塞造成的，而非肝静脉和下腔静脉的闭塞。

HCT后肝脏SOS的预防和处理将总结在此。HCT后SOS的发病机制、临床特征和诊断将单独讨论。 (参见“造血干细胞移植后肝窦阻塞综合征(肝小静脉闭塞病)的诊断”)

预防

某些移植前特征和移植过程中相关的因素同SOS的发生有关。这些相关性的强度在不同研究中有所不同，没有独立的因素或联合的因素可以解释患者SOS风险的差异(表 1)。

有些移植前特征同较高的SOS风险有关，包括已有肝脏疾病[血清天冬氨酸氨基转移酶(aspartate aminotransferase, AST)升高]、患者较年轻(<7岁的儿童发生率较高)，以及基线日常体能状态较差。已提出的移植过程相关SOS危险因素包括：移植物来源(异基因移植较自体移植风险高)、预处理方案选择(使用环磷酰胺及大剂量放疗的SOS风险更高，使用降低强度方案的SOS风险更低)、移植物抗宿主病(graft-versus-host disease, GVHD)预防治疗的选择(西罗莫司联合某些预处理方案)，以及移植前特定抗生素的使用(如，万古霉素、两性霉素和阿昔洛韦)。这些将单独更详细地介绍。 (参见“造血干细胞移植后肝窦阻塞综合征(肝小静脉闭塞病)的诊断”，关于‘概述’一节)

                 

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Literature review current through: 2017-06 . | This topic last updated: 2016-04-01.
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