肝淀粉样变性临床及影像分析  

Clinical and imaging analysis of hepatic amyloidosis

作　　者：郝磊 任洪伟 董景辉 蔡剑鸣 张萌萌 刘长春 高珅 刘渊 HAO Lei;REN Hong-wei;DONG Jing-hui;CAI Jian-ming;ZHANG Meng-meng;LIU Chang-chun;GAO Shen;LIU Yuan(Department of Radiology,Hepingli Hospital,Beijing 100013,China;Department of Radiology,Fifth Medical Center of PLA General Hospital,Beijing 100039,China)

机构地区：[1]和平里医院放射科,北京市100013 [2]解放军总医院第五医学中心放射诊断科,北京100039

出　　处：《肝脏》2023年第11期1359-1362,共4页Chinese Hepatology

基　　金：“首都临床特色应用研究”专项(Z181100001718006);首都卫生发展科研专项项目(首发2022-4-5061)支持

摘　　要：目的探讨分析肝淀粉样变性的临床及影像学特点,为临床提供经验和参考。方法回顾性分析解放军总医院第五医学中心2017年2月—2023年6月期间收治的12例经病理活检证实的肝淀粉样变性患者的各项资料,归纳分析患者的一般资料、临床表现、实验室检验和影像学表现等。结果12例肝淀粉样变性患者的临床表现主要为腹胀、纳差、发现肝功能异常,入组患者中碱性磷酸酶升高者10例,谷氨酰转肽酶升高者11例,白蛋白降低者7例,肿瘤标志物全呈阴性者5例,CA125升高者4例,CA199升高者6例。12例均有肝脏体积增大,7例脾脏增大,7例有腹腔积液,9例增强扫描门静脉期及延迟期肝实质内可见“窗凌花”样斑片状低密度或低信号强化影。结论肝淀粉样变性多见于中老年女性,主要临床表现为腹胀、纳差和肝功能异常,大部分患者可有碱性磷酸酶和谷氨酰转肽酶升高,部分肿瘤标志物异常,影像学多表现为肝脾增大,腹水和肝实质强化不均匀,可有类似“窗凌花”样强化,确诊肝淀粉样变性的主要手段是影像学表现结合肝穿刺活检病理结果。Objective To analyze the clinical and imaging features of 12 patients with hepatic amyloidosis.Methods Data from 12 patients who had undergone pathological biopsies between February 2017 and February 2023 were collected and analyzed.General demograpgics,clinical findings,laboratory results and imaging findings were included in this study.Results In 12 patients diagnosed with hepatic amyloidosis,the predominant clinical manifestations included abdominal distension,poor appetite,and abnormal liver function.Among these patients,10 showed elevated alkaline phosphatase(ALP),11 had elevated glutamyltransferase(GGT),7 exhibited decreased albumin(ALB),5 tested negative for tumor markers,4 had elevated CA125,and 6 indicated elevated CA199 levels.Hepatomegaly was evident in all 12 patients,Additionally,an enlarged spleen was identified in 7 patients,abdominal effusion in 7,and 9 displayed a“frozen snowflakes on window”-like patchy low-density or low signal enhancement in the liver parenchyma during both the portal and delayed phases.Conclusion Hepatic amyloidosis predominantly affects middle-aged and elderly women.The primary clinical symptoms include abdominal distension and abnormal liver function.Most patients exhibit elevated levels of ALP and GGT,with some showing abnormal some tumor markers.The most frequent imaging findings are enlargement of the liver and spleen,ascites,heterogeneous liver parenchymal enhancement,and enhancement patteins reminiscent of“frozen snowflakes on a window”.The definitive diagnosis of hepatic amyloidosis relies on a combination of imaging results and pathological findings from liver puncture biopsy.

关 键 词：肝淀粉样变性 磁共振 体层摄影术 扩散加权成像 

分 类 号：R575[医药卫生—消化系统]