Clinicopathological analysis of 11 cases of hepatic amyloidosis
10.3760/cma.j.cn501113-20210107-00011
- VernacularTitle:11例肝淀粉样变性的临床病理分析
- Author:
Yuanyuan REN
1
;
Chen SHAO
;
Ming ZHANG
;
Yujiao ZHANG
;
Lichao YUAN
;
Xinzhen GUO
;
Jing ZHANG
;
Li ZHOU
;
Xiuxia LI
;
Anlin MA
;
Tailing WANG
Author Information
1. 中日友好医院感染疾病科,北京 100029
- Keywords:
Liver;
Amyloidosis;
Jaundice
- From:
Chinese Journal of Hepatology
2022;30(11):1207-1210
- CountryChina
- Language:Chinese
-
Abstract:
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 and γ-glutamyl transferase levels were significantly raised in all cases, with the highest result for γ-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.
