摘要
目的:评估肝脏系统性轻链型淀粉样变性(AL淀粉样变性)的临床特点和预后影响因素。方法:回顾性分析81例肝脏AL淀粉样变性患者的临床及随访资料。结果:患者中位年龄55岁,男女比例2.9∶1。主要临床表现包括水肿、消化系统症状、体重下降、乏力和腹水等。51例患者接受治疗,34例可进行疗效评估,其中19例(55.9%)取得血液学缓解,未取得血液学缓解患者的死亡风险是取得血液学缓解患者的6.6倍。中位生存期9个月,1年、3年、5年生存率分别为31.0%、17.8%和10.7%,多因素分析表明基线N端前脑钠肽≥1800 pg/ml是预后独立危险因素。结论:肝脏AL淀粉样变性临床表现多样,预后差,N端前脑钠肽显著升高提示预后不良,对相关临床表现提高警惕,早期诊断、及时治疗可改善预后。
Objective:To evaluate clinical manifestation and prognosis of hepatic systemic light chain(AL)amyloidosis.Methodology:We retrospectively analyzed the clinical data and outcomes of 81 patients diagnosed with hepatic AL amyloidosis.Results:The median age was 55,with a male to female ratio of 2.9∶1.The main clinical manifestations included edema,digestive symptoms,weight loss,fatigue and ascites.Among 51 patients who received treatment,the therapeutic effect could be evaluated in 34 patients.19 patients(55.9%)achieved hematological remission,and the risk of death in patients who did not achieve hematological remission was 6.6 times higher than that in patients achieved hematological remission.The median survival time was 9 months,with a 1,3 and 5-year survival rate of 31.0%,17.8%and 10.7%,respectively.Multivariate analysis showed that baseline NT-proBNP≥1800 pg/ml was an independent risk factor for prognosis.Conclusion:The clinical manifestations of hepatic AL amyloidosis are diverse and the prognosis is poor.The significantly elevated NT-proBNP indicates poor outcome.Early diagnosis and timely treatment may improve the prognosis.
作者
赵亮
任贵生
郭锦洲
陈文萃
陈轻获
刘志红
黄湘华
ZHAO Liang;REN Guisheng;GUO Jinzhou;CHEN Wencui;CHEN Qinghuo;LIU Zhihong;HUANG Xianghua(National Clinical Research Center of Kidney Diseases,Jinling Hospital,Nanjing University School of Medicine,Nanjing 210016,China)
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
北大核心
2019年第4期318-323,共6页
Chinese Journal of Nephrology,Dialysis & Transplantation
基金
江苏省重点研发计划社会发展面上项目(BE2017721)
江苏省自然科学基金青年基金项目(BK20170625)
关键词
肝脏淀粉样变性
轻链型淀粉样变性
临床表现
预后
hepatic amyloidosis
light chain amyloidosis
clinical manifestation
prognosis
