肝淀粉样变性与肝窦阻塞综合征临床及影像特征的鉴别分析

Clinical and imaging identification between hepatic amyloidosis and hepatic sinusoidal obstructive syndrome

目的肝淀粉样变性与肝窦阻塞综合征的病理发病部位同为肝窦周隙,影像学表现相似,临床表现又缺乏特异性,临床对其认识不足,容易误诊,本研究通过总结肝淀粉样变性及肝窦阻塞综合征的临床及影像特点,提高对两种疾病的诊断水平。材料与方法回顾北京佑安医院2009-2019年经病理诊断的11例肝淀粉样变性和20例肝窦阻塞综合征患者的临床及影像资料,比较其差异。结果(1)肝淀粉样变性的典型临床特征是巨肝、肝功能损害轻微但碱性磷酸酶(alkaline phosphatase,ALP)和谷氨酰转肽酶(γ-glutamyl transpeptadase,GGT)显著升高,国内肝窦阻塞综合征患者多有服用土三七病史,临床多以黄疸伴肝功能受损、凝血功能障碍及糖类抗原125(carbohydrate antigen 125,CA125)增高为主要表现。(2)影像学检查:①超声肝弹性成像检查中肝淀粉样变性肝实质硬度极高(≥75 kPa),显著高于肝窦阻塞综合征患者的肝脏硬度[(44.65±19.01)kPa];②MRI上肝淀粉样变性T2WI像肝实质信号均匀、细腻,肝“纹理”减少;增强扫描肝实质强化程度减低且强化峰值延迟,静脉期肝实质呈“窗凌花”样改变,可同时伴有脾脏或肾脏的低血流灌注征象。而肝窦阻塞综合征T2WI像肝实质信号不均匀增高,可见不规则片状或呈“云絮”状稍高信号影;增强扫描门脉期肝实质可呈现地图样强化及环绕三支肝静脉的“三叶草”样强化,延迟期肝实质强化范围较门脉期进一步增大;③肝淀粉样变性肝动脉及门脉主干管径大于肝窦阻塞综合征患者(P<0.05);两者病例影像的共同点是静脉期三支肝静脉不显影或显影纤细。结论肝淀粉样变性与肝窦阻塞综合征的病因、临床表现及影像学表现均存在一定的差别,可利用这些差别进行鉴别诊断。

Objective:The pathological site of hepatic amyloidosis and hepatic sinusoidal obstruction syndrome is the space of disse.The imaging findings can be similar,but the clinical manifestations lack specificity.It is easy to be misdiagnosed due to lack of clinical understanding.To summarize the clinical and imaging characteristics of hepatic amyloidosis and hepatic sinusoidal obstruction syndrome,and to improve the diagnosis of the two diseases.Materials and Methods:Reviewed the clinical and imaging data of 11 patients with hepatic amyloidosis and 20 patients with hepatic sinusoidal obstruction syndrome diagnosed pathologically in Beijing You'an Hospital from 2009 to 2019,and compared their differences.Results:(1)The typical clinical features of hepatic amyloidosis were giant liver,slight liver damage but significant increased in ALP and GGT.Most patients with hepatic sinusoidal obstruction syndrome in China had taken tunotoginseng history.The clinical features were mostly jaundice with liver.Functional impairment,coagulation dysfunction and increased CA125 were the main manifestations.(2)Imaging examination:①The stiffness of liver amyloidosis on ultrasound was extremely high(≥75 kPa),which was significantly higher than that of sinusoidal obstruction syndrome[(44.65±19.01)kPa];②Hepatic amyloidosis on MRI T2WI,the liver parenchymal signal was uniform and fine,and the liver"texture"was reduced;the enhanced liver parenchymal enhancement was reduced and the enhancement peak was delayed.The liver parenchyma in the venous phase shows"windows Linghua"-like changes,which might be accompanied by low blood flow in the spleen or kidneys.Signs of perfusion,on the other hand,the T2WI liver parenchymal signal of hepatic sinus obstruction syndrome was unevenly increased,showing irregular patches or"cloudy"-like slightly high signal shadows;enhanced scan portal phase liver parenchyma could show map-like enhancement,surrounding three hepatic veins"Clover"-like enhancement,the delayed phase enhancement range was enlarged;③Amyloidosis,the hepatic artery and the main portal vein diameter were larger than the hepatic sinusoidal obstruction syndrome patients(P<0.05);the common point of the images of the two cases was the venous phaseⅢ.The branch hepatic veins were not visible or appear slender.Conclusions:There are some differences on the cause,clinical characteristic,and characteristic of images between hepatic amyloidosis and hepatic sinusoidal obstructive syndrome,all of them contribute to differential diagnosis.