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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

Authors
Robert S Negrin, MD
Peter A L Bonis, MD
Section Editor
Nelson J Chao, MD
Deputy Editor
Alan G Rosmarin, MD
Translators
吴德沛, 主任医师，教授

引言

肝窦阻塞综合征(sinusoidal obstruction syndrome, SOS)，以前称为肝小静脉闭塞病(veno-occlusive disease, VOD)，特征为肝肿大、右上腹痛、黄疸和腹水，最常见于接受造血干细胞移植(hematopoietic cell transplantation, HCT)的患者，少数情况下也可见于非移植情况下使用化疗药物后、摄入生物碱毒素后、高剂量放射治疗或肝移植后。该病在临床上与巴德-吉亚利综合征相似；但SOS中肝静脉流出道梗阻是由终末肝微静脉或肝窦闭塞，而非肝静脉或下腔静脉闭塞所致。

HCT后SOS的发病机制、临床特点和诊断将总结在此。而该病的预防和治疗将单独讨论。 (参见“造血干细胞移植后肝窦阻塞综合征的治疗和预防”)

本专题会将“造血干细胞移植(HCT)”这个词作为涵盖任何来源(例如，骨髓、外周血、脐血)祖细胞移植的一般术语通篇使用。除此以外，这些细胞的来源(例如，自体外周血祖细胞移植)将会具体说明。 (参见“造血干细胞的来源”)

发病机制

SOS通常发生于HCT的情况下。然而，其他因素亦可能诱发该疾病，包括：摄入吡咯里西啶生物碱，常来源于草药(如灌木茶和其他药草茶)[1]；对肝脏进行高剂量放射治疗(通常超过30Gy)不伴减瘤性化疗[2,3]；肝肿瘤的放射性栓塞术[4]；以及肝移植后[5,6]。 (参见“草药和膳食补充剂的肝毒性”)

无论何种病因，一般认为SOS开始于肝静脉内皮细胞损伤。已存在肝脏疾病会导致SOS发生的风险增加，这一现象为该疾病的发病机制提供了一些线索。已存在肝脏疾病可能会损害药物的代谢，因此易发生内皮细胞损伤。此外，慢性肝炎患者的肝脏内皮细胞可能存在异常，这会使其更易受到减瘤性治疗方案的影响。肝炎患者的内皮细胞可能会异常表达黏附分子和促凝血因子[7-9]。

                   

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Literature review current through: 2017-06 . | This topic last updated: 2017-03-30.
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