菊三七相关肝窦阻塞综合征115例的临床特征分析

Clinical characteristics of 115 cases of gynura segetum induced hepatic sinusoidal obstruction syndrome

目的了解菊三七相关肝窦阻塞综合征（HSOS）的主要临床特征。方法回顾性分析2008年7月至2016年10月因服用菊三七导致HSOS患者115例，记录患者发病前服用菊三七及其他药物史，观察主要临床症状的流行病学资料、临床表现、实验室检查、影像学和病理表现。结果115例患者中，腹部胀痛113例，纳差106例，巩膜黄染42例。血清TBil升高108例，其中41例为轻度升高；29例白蛋白≤30 g/L；64例PT延长，11例延长超过3 s；31例活化部分凝血活酶时间延长；60例PLT计数低于正常值。92例行超声检查，肝脏体积增大71例，肝脏内回声不均匀79例，腹水70例，肝静脉周围组织可见片状低回声区，呈斑片状14例。60例行CT检查，均发现有腹水，14例轻度脾大，8例食管胃底静脉曲张；平扫示肝肿大及肝脏密度减低、延迟期间斑片状低密度呈＂地图样＂改变，动脉期肝实质内斑片样不均匀强化，下腔静脉肝段受压变形和静脉期门静脉周围晕征。病理检查示肝窦扩张伴出血及淤血，小叶Ⅲ区肝板结构破坏。7例患者行肝静脉压力梯度测量，均显著升高。结论菊三七所致HSOS可表现为腹部胀痛、纳差、黄疸；TBil轻度升高，白蛋白轻度降低；肝脏肿大、门静脉、脾静脉血流速度减慢；肝静脉压力梯度明显升高，肝窦淤血、Ⅲ区肝细胞凝固性坏死等。

Objective To study the clinical characteristics of gynura segetum induced hepatic sinusoidal obstruction syndrome （HSOS）. Methods From July 2008 to October 2016, a total of 115 cases of gynura segetum caused HSOS were retrospectively analyzed. The history of taking gynura segetum before disease onset was recorded and epidemiologic data of main clinical symptoms, clinical manifestations, laboratory examinations, imaging and pathological features were observed. Results Among the 115 cases of HSOS, there were 113 patients with abdominal pain, 106 with anorexia and 42 with jaundice sclera. A total of 108 patients displayed increased serum total bilirubin, 41 of them only with mildly increased total bilirubin. There were 29 patients with albumin lower than 30 g/L, 64 patients with prolonged protbrombin time （PT） and PT of 11 patients was prolonged for more than three seconds. Meanwhile, 31 patients were with prolonged activated partial prothrombin time （APTT）. A total of 60 patients had low platelet count. And 92 patients underwent ultrasound examination, among them, 71 patients had enlarged liver size, 79 patients with uneven internal echo of liver, 70 patients with ascites, 14 patients with patchy low echo tissue around hepatic venous. A total of 60 patient accepted computed tomography （CT） examination, and all of them had ascites, 14 patients with mildly enlarged spleen and eight patients with gastro-esophageal varices. The results of CT plain scan indicated hepatomegaly, decreased liver density, ＂map-like＂ changes of patchy low density in delayed phase, heterogeneous enhancement of liver parenchyma in arterial phase, compression and deformation of liver segment of inferior vena cava and halo sign around venous portal vein. The results of pathological examination demonstrated the widening of hepatic sinusoid with hemorrhage and congestion, destruction of liver plate in zone Ⅲ area. There were seven patients who received hepatic venous pressure gradient （HVPG） measurement which were all significantly increased. Conclusions The characteristics of patients with gynura segetum caused HSOS are abdominal pain, anorexia and jaundice; mildly increased serum total bilirubin and albumim liver enlargement, slow blood velocity of portal vein and splenic veim increased HVPG, hepatic sinus congestion and cell coagulation necrosis in zoneⅢ area.