肝窦阻塞综合征35例临床分析及文献复习

Clinical features of sinusoidal obstruction syndrome: an analysis of 35 cases and literature review

目的总结肝窦阻塞综合征的临床特点、诊断和治疗方法并复习文献,旨在提高临床对该疾病的诊疗水平。方法回顾性分析2008年8月-2013年5月在南京医科大学第一附属医院住院的35例患者病史、临床表现、实验室结果以及影像学特征、肝脏组织病理学、治疗经过及预后情况。结果 31例患者有服用土三七病史,占88.6%;2例有服用其他中药史;2例有异基因造血干细胞移植史。临床表现主要为腹胀(100%)、腹水(91.4%)、腹痛(62.9%)、黄疸(48.6%),不同程度的肝功能损害。超声检查发现肝静脉显示不清或明显变细,血流降低或消失;CT发现肝脏呈"地图状"强化不均匀;肝脏组织病理学发现局部肝窦扩张,部分肝细胞脂肪变性,部分肝组织出血,肝组织及门静脉区大量慢性炎症细胞浸润。治疗后,15例(42.9%)好转,20例(57.1%)治疗无效自动出院。结论服用土三七仍为我国SOS的主要病因,早期诊断,及时应用改善肝脏微循环药物以及糖皮质激素对改善预后有一定疗效。

Objective To summarize the clinical features, diagnosis, and treatment of sinusoidal obstruction syndrome （SOS） and review relevant literature and to improve the clinical diagnosis of this disease. Methods A retrospective analysis was performed on the medical his- tories, clinical manifestations, laboratory results, imaging findings, liver histopathology, treatments, and prognosis of 35 patients with SOS who were hospitalized at the First Affiliated Hospital of Nanjing Medical University from August 2008 to May 2013. Results Of all patients, 31 （ 88.6% ） had taken Gynura segetum, 2 had received other traditional Chinese medicines, and 2 had undergone allogeneic hematopoietic stem cell transplantation. The main symptoms included abdominal distention （ 100% ）, ascites （91.4%）, hepatalgia （62.9%）, and jaun- dice （48.6%）, as well as varying degrees of liver function damage. Ultrasound examination revealed unclear or thinner hepatic veins and slower or no blood flow. CT scans showed map - like heterogeneous enhancement in the liver. Pathological examination demonstrated local sinusoidal congestion, steatosis of some hepatocytes, hemorrhage in some liver tissue, and massive chronic inflammatory cell infiltration in the liver tissue and portal area. After treatment, 15 cases （42.9%） showed improvement, and 20 cases （57.1%） showed no response and were discharged. Conclusion Taking Gynura segetum is still the main cause of SOS in China. Early diagnosis and timely use of drugs for improving hepatic microcirculation and glucocorticoids are effective in improving the prognosis of SOS.