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门静脉-肝窦血管性疾病与肝窦阻塞综合征：同一疾病的不同阶段？
DOI: 10.12449/JCH260404
利益冲突声明：本文不存在任何利益冲突。
作者贡献声明：王春香、梁思薇负责文献分析，论文撰写；张峰、诸葛宇征负责论文修改，指导写作思路。
Porto-sinusoidal vascular disorder and hepatic sinusoidal obstruction syndrome： Different stages of the same disease？
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摘要: 门静脉-肝窦血管性疾病（PSVD）与肝窦阻塞综合征（HSOS）同属肝脏血管性疾病，二者病因有所不同，临床表现不同，治疗和预后亦不同。然而，近年来的研究证据逐渐表明，PSVD与HSOS之间存在尚未被充分认识的关联，提示二者可能是同一疾病的不同表现形式。本文着重对二者的关联及相关研究证据进行综述。
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关键词:
- 门静脉-肝窦血管性疾病 /
- 肝窦阻塞综合征 /
- 病理状态， 体征和症状
Abstract: Both porto-sinusoidal vascular disorder and hepatic sinusoidal obstruction syndrome belong to hepatic vascular diseases， and they have differences in etiology， clinical manifestations， treatment， and prognosis. However， recent research evidence has gradually shown that the association between porto-sinusoidal vascular disorder and hepatic sinusoidal obstruction syndrome has not been fully understood， suggesting that they may be two different manifestations of the same disease. This article reviews the association between these two diseases and related research evidence. -
注： HSOS，肝窦阻塞综合征；PSVD，门静脉-肝窦血管性疾病。
图 1 奥沙利铂导致的HSOS和PSVD特征比较
Figure 1. Comparison of HSOS and PSVD characteristics induced by oxaliplatin
表 1 PSVD与HSOS的区别
Table 1. Differences between PSVD and HSOS
项目 PSVD HSOS 诱因 免疫系统疾病、凝血功能异常、遗传性疾病、慢性感染、
细菌移位及药物毒物接触史含吡咯生物碱药物、化疗药物、造血干细胞移植等 起病 缓慢且隐匿 急性 典型表现 早期无症状，进展后出现门静脉高压并发症 表现为急性门静脉高压 肝功能特征 肝功能多正常或轻度异常 肝功能明显受损 门静脉高压类型 窦前/窦性为主，HVPG正常或轻度升高 窦性/窦后型为主，HVPG多>10 mmHg 病理特征 门静脉狭窄、结节性再生性增生、不完全间隔纤维化，
无肝硬化假小叶肝窦内皮细胞肿胀、损伤、脱落，肝窦显著扩张充血 注：PSVD，门静脉-肝窦血管性疾病；HSOS，肝窦阻塞综合征；HVPG，肝静脉压力梯度。
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