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  

Analysis of clinical manifestations and prognosis of primary systemic light chain amyloidosis with liver involvement

作　　者：乔彤彤 刘扬[1] 彭楠 宫立众 窦雪琳 温磊[1] 路瑾[1] Qiao Tongtong;Liu Yang;Peng Nan;Gong Lizhong;Dou Xuelin;Wen Lei;Lu Jin(Department of Hematology,Peking University People's Hospital,Peking University Institute of Hematology National Clinical Research Center for Hematologic Disease,Beijing 100044,China;Hebei Provincial Traditional Chinese Medicine Hospital,Shijiazhuang 050033,China)

机构地区：[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 [2]河北省中医院血液科,石家庄050033

出　　处：《中华肝脏病杂志》2024年第3期222-227,共6页Chinese Journal of Hepatology

基　　金：国家自然科学基金(82170196)。

摘　　要：目的总结单中心肝脏淀粉样变性患者的临床表现及其预后相关因素。方法回顾性分析2012年10月至2023年1月期间的28例明确诊断为伴肝脏受累的原发系统性轻链型淀粉样变性患者的临床资料,总结其主要临床表现和预后因素;据数据不同采用χ2检验、Fisher精确检验,Wilcoxon秩检验或Kaplan-Meier生存曲线对数秩检验进行统计学分析。结果肝脏受累患者临床主要表现为腹胀、肝大、水肿。CD56、趋化因子受体4蛋白表达占52%(13/25)、56%(14/25),64.3%(9/14)患者t(11,14)阳性,21.4%(3/14)患者1q21扩增阳性,无患者检测出del(17p)。单因素分析显示梅奥2004、2012分期以及总胆红素(TBil)≥34.2μmol/L与患者无进展生存及总体生存相关。TBil≥34.2μmol/L组患者中位无进展生存及总生存期(0.178年及0.195年)明显短于TBil<34.2μmol/L组(0.750年及3.586年)(P<0.05)。结论梅奥分期、高胆红素血症是原发系统性轻链型淀粉样变性伴肝脏受累患者的预后不良因素。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)≥34.2μmol/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<0.05).Conclusion Mayo stage and hyperbilirubinemia are inferior prognostic factors for patients with primary systemic light chain amyloidosis accompanied with liver involvement.

关 键 词：淀粉样变性 肝脏 总胆红素 预后分析 

分 类 号：R575[医药卫生—消化系统]