Analysis of clinical manifestations and prognosis of primary systemic light chain amyloidosis with liver involvement
10.3760/cma.j.cn501113-20231108-00185
- VernacularTitle:肝脏受累原发系统性轻链型淀粉样变性的临床表现及其预后分析
- Author:
Tongtong QIAO
1
;
Yang LIU
;
Nan PENG
;
Lizhong GONG
;
Xuelin DOU
;
Lei WEN
;
Jin LU
Author Information
1. 北京大学人民医院血液科 北京大学血液病研究所 国家血液系统疾病临床医学研究中心，北京 100044
- Keywords:
Amyloidosis, liver;
Total bilirubin;
Prognosis
- From:
Chinese Journal of Hepatology
2024;32(3):222-227
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To summarize the clinical manifestations and prognostic factors of patients with hepatic amyloidosis in a single center.Methods:The clinical data of 28 primary systemic light chain amyloidosis cases with liver involvement in our center from October 2012 to January 2023 were retrospectively analyzed. The main clinical manifestations and prognostic factors were studied. Statistical analysis were performed using the χ 2 test, Fisher's exact test, Wilcoxon rank test, or Kaplan-Meier survival curve log-rank test according to the different data. Results:The main clinical manifestations of patients with liver involvement were abdominal distension, hepatomegaly, and edema. CD56 and chemokine receptor 4 protein expression accounted for 52% (13/25) and 56% (14/25). 64.3% (9/14) patients were combined with t (11,14), and 21.4% (3/14) patients were positive for 1q21 (+), and no patients were detected with del(17p). Univariate analysis showed that Mayo 2004 and 2012 stages and total bilirubin (TBil) ≥34.2 μmol/L were associated with progression-free survival and overall survival. The median progression-free survival and overall survival were significantly inferior in patients with TBil≥34.2μmol/L group (0.178 years, 0.195 years) than with the TBil<34.2μmol/L group (0.750 years, 3.586 years) ( P ?
