11例肝淀粉样变性的临床病理分析  

Clinicopathological analysis of 11 cases of hepatic amyloidosis

作　　者：任媛媛 邵晨 张明[1] 张玉姣[1] 袁利超[1] 郭新珍[1] 张璟[1] 周利 李秀霞 马安林[1] 王泰龄[3] Ren Yuanyuan;Shao Chen;Zhang Ming;Zhang Yujiao;Yuan Lichao;Guo Xinzhen;Zhang Jing;Zhou Li;Li Xiuxia;Ma Anlin;Wang Tailing(Department of Infectious Disease,China-Japan Friendship Hospital,Beijing 100029,China;Department of Pathology,Beijing You An Hospital,Beijing 100029,China;Department of Pathology,China-Japan Friendship Hospital,Beijing 100029,China)

机构地区：[1]中日友好医院感染疾病科,北京100029 [2]北京佑安医院病理科,北京100029 [3]中日友好医院病理科,北京100029

出　　处：《中华肝脏病杂志》2022年第11期1207-1210,共4页Chinese Journal of Hepatology

摘　　要：目的肝脏淀粉样变性是一种发病率低的代谢性疾病,因发病隐匿,误诊率较高,且确诊时一般已进展至较晚期。现分析肝淀粉样变性的临床特征,以期临床病理相结合,提高临床诊断率。方法总结并回顾性分析中日友好医院2003年至2017年诊断的11例肝脏淀粉样变患者的临床及病理资料。结果11例患者的临床表现主要包括腹部不适(4/11),肝大(7/11),脾肿大(5/11),乏力(6/11)等。生物化学检测结果显示丙氨酸转氨酶、天冬氨酸转氨酶,胆管酶碱性磷酸酶、γ-谷氨酰转移酶升高,大部分患者总胆红素、直接胆红素、总胆汁酸升高,并伴有低白蛋白血症。部分患者24h尿蛋白定量、肌酐、尿素氮升高。结论患者均有天冬氨酸转氨酶轻度升高,在正常值上限5倍以内,72%患者丙氨酸转氨酶也轻度升高。患者均有胆管酶碱性磷酸酶、γ-谷氨酰转移酶明显升高,γ-谷氨酰转移酶最高可达正常值上限的51倍。除了累及胆道系统,肝细胞的损伤最终导致门静脉高压,低白蛋白血症[(0.54~0.63)×正常值上限,9/11]。血管损伤方面,54.5%的患者动脉壁及36.4%的患者门静脉也可见明显的淀粉样物沉积。出现不明原因转氨酶、胆管酶升高、门静脉高压的患者,应建议肝穿刺明确诊断。Objective Hepatic amyloidosis is a metabolic disease with a low incidence rate.However,because of its insidious onset,the rate of misdiagnosis is high,and it usually progresses to a late stage when it is diagnosed.This article analyzes the clinical features of hepatic amyloidosis by combining clinical pathology in order to improve the clinical diagnosis rate.Methods Clinical and pathological data of 11 cases of hepatic amyloidosis diagnosed at the China-Japan Friendship Hospital from 2003 to 2017 were summarized and analyzed retrospectively.Results The clinical manifestations of 11 cases mainly included abdominal discomfort(4/11),hepatomegaly(7/11),splenomegaly(5/11),fatigue(6/11),etc.Biochemical test results showed that most patients'alanine aminotransferase,aspartate aminotransferase,alkaline phosphatase,γ-glutamyl transferase,total bilirubin,direct bilirubin,and total bile acids,accompanied by hypoalbuminemia were elevated,while some patients'24-h urinary protein,creatinine,and blood urea nitrogen were elevated.Conclusion All patients had slightly elevated aspartate transaminase levels(within 5 times the upper limit of normal),and 72%had slightly elevated alanine transaminase.Alkaline phosphatase andγ-glutamyl transferase levels were significantly raised in all cases,with the highest result forγ-glutamyl transferase being 51 times the upper limit of normal.Damage to the hepatocytes has an effect on the biliary system as well,leading to symptoms such as portal hypertension and hypoalbuminemia[(0.54~0.63)×upper limit of normal value,9/11].Amyloid deposits within the artery wall(54.5%of patients)and portal vein(36.4%of patients)were also indicative of vascular injury.A liver biopsy should be recommended for patients with unexplained elevated transaminases,bile duct enzymes,and portal hypertension in order to establish a definitive diagnosis.

关 键 词：肝脏 淀粉样变 黄疸 

分 类 号：R575[医药卫生—消化系统]