肝窦阻塞综合征的临床分析

目的 总结肝窦阻塞综合征（hepatic sinusoidal obstruction syndrome，HSOS） 的临床特点，提高对该病的认识。 方法 回顾性分析解放军总医院23 例HSOS 的临床资料。 结果 23 例中8 例（34.7%） 近期曾服用中药（ 其中4 例含有土三七），2 例（8.7%） 接受化疗及免疫抑制剂治疗，2 例（8.7%） 服用镇痛药（ 含非甾体类药物），11 例（47.9%） 无明确诱因。临床主要表现为腹水（78.3%）、腹胀（73.9%）、肝大（21.7%）、肝区疼痛（26.1%）、黄疸（17.4%），有不同程度的肝功能受损。B 超检查见肝质地回声增粗、分布不均，呈“斑片状”，肝静脉显示不清或明显变细，血流降低或消失，肝段下腔静脉多表现为受压变细；增强CT 示肝脏呈“地图状”强化不均匀；血管造影显示肝静脉远端小分支呈“枯树枝样”或“蜘蛛网样”改变；病理学表现为肝窦淤血、扩张，肝细胞不同程度变性、坏死，中央静脉或小叶下静脉内皮水肿或纤维增生。经内科治疗18 例（78.3%）好转，1 例（4.3%） 未治，2 例（8.7%） 无效，2 例（8.7%） 死亡。 结论 服用某些中药、化疗及免疫抑制剂治疗是主要诱因，确诊需要病理依据，根据病史、临床表现及特征性的影像学表现及病理结果可临床诊断，早期使用保肝、抗凝、改善微循环药物对改善预后有一定作用。

Clinical features of hepatic sinusoidal syndrome

Objective To improve the understanding of diagnosis and treatment of hepatic sinusoidal syndrome（HSOS） by analyzing its clinical features. Methods Clinical data about 23 HSOS patients admitted to Chinese PLA General Hospital were retrospectively analyzed. Results Of the 23 HSOS patients, 8（34.7%） were treated with Chinese medicines containing Gynura Rhizom, 2（8.7%） received chemotherapy and immunosuppressive therapy, 2（8.7%） were given analgesics including nonsteroidal anti-inflammatory agents, and 11（47.9%） had no clear predisposing cause. Their main clinical manifestations were ascites（78.3%）, abdominal distension（73.9%）, hepatomegaly（21.7%）, abdominal pain at the right upper quadrant（26.1%） and jaundice（17.4%）, with varying degrees of impaired liver function. Type-B ultrasonography（US） showed uneven distribution of enhanced echo in liver, unclear plaque or very fine hepatic vein , decreased or disappeared blood flow in portal vein , and flat posterohepatic vena cava. Enhanced CT scanning showed map-like uneven distribution of contrast agent in liver. Angiography revealed dry branch like or spider weblike changes in liver. Pathology demonstrated hepatic sinusoid congestion and dilatation, degeneration and necrosis of hepatic cells at different degrees, endothelial oedema or fibrosis of central vein or sublobular vein. Of the 23 patients after medical treatment , 18（78.3%） were improved, 1（4.3%） received no treatment, 2（8.7%） failed to treatment, and 3（8.7%） died. Conclusion Take administration of certain Chinese herbal medicine , chemotherapy and immunosuppressive therapy are the main predisposing cause of HSOS. Its diagnosis can be established according to its pathology, history, clinical manifestations and characteristic imaging. Early use of liver-protective, anticoagulant and microcirculation drugs can improve the prognosis of HSOS patients.