轻链淀粉样变性肾病的临床特点及生存分析

中国医师进修杂志 Pub Date : 2010-11-05 DOI:10.3760/CMA.J.ISSN.1673-4904.2010.31.003

Wang Hehua, Wen-fang Chen, X. Tong, Mian-sheng Yan, Duo-rong Xu, L. Juan

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引用次数: 1

摘要

目的分析轻链淀粉样变性肾病的临床病理特点，探讨影响患者生存的因素。方法对1998年1月~ 2009年3月25例轻链淀粉样变性肾病患者进行回顾性分析和随访。结果25例轻链淀粉样变性肾病患者中位年龄为57(37 ~ 69)岁，以lamda轻链为主(88%，22/25)。重度蛋白尿和肾病综合征伴周围水肿是典型的临床表现。肾活检显示轻链淀粉样变性相关肾病的淀粉样沉积主要累及肾小球，伴系膜区增宽。所有患者的中位生存期为诊断后24.4个月。估计1、2、3年生存率分别为(65±10)%、(46±12)%和(15±12)%。两组患者的中位生存期差异有统计学意义(14例孤立肾受累组为24.7个月，11例肾脏及其他脏器受累组为16.4个月，P = 0.03)。单因素分析显示，肾脏合并其他脏器淀粉样变性和肾功能不全与预后相关(P < 0.05)，可能与心脏受累相关(P = 0.06)，而性别、年龄、浆细胞比、血清白蛋白水平和血红蛋白水平与预后无关(P < 0.05)。多因素分析显示，诊断时肾功能不全是影响患者生存的重要独立预后因素(P <0.05)。结论诊断时肾功能不全是预测患者生存的最佳指标。在肾脏以外的器官出现淀粉样变，如晚期心脏淀粉样变，预示着较差的生存率。关键词:预后;肾淀粉样变;免疫球蛋白轻链;生存分析

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Clinical features and survival analysis of light-chain amyloidosis associated renal disease

Objective To analyze the clinical pathology features of light-chain amyloidosis associated renal disease,and investigate the survival influential factors. Method From January 1998 to March 2009,25 patients with light-chain amyloidosis associated renal disease were reviewed and followed up.Results Of the 25 patients with light-chain amyloidosis associated renal disease,median age was 57(37-69) years old and lamda light-chain predominated (88% ,22/25). Heavy proteinuria and nephrotic syndrome with peripheral edema were typical clinical presentations. Renal biopsy showed that amyloid deposition of light-chain amyloidosis associated renal disease involved the glomeruh mostly, with mesangial area widening. Median survival of all patients was 24.4 months after diagnosis. The estimated 1,2,3 year survival rate was (65 ± 10 )%, (46 ± 12 )% and (15 ± 12 )% respectively. There was significant difference in median survival between the two groups (24.7 months in the group of 14 patients with isolated kidney affected,16.4 months in the group of 11 patients with kidney and other organs involved,P = 0.03). By univariate analysis, kidney associated with other organs amyloidosis and renal dysfunction were relevant to prognosis (P ＜ 0.05) and heart involvement was probably relevant (P = 0.06),whereas sex,age,plasma cell ratio,serum albumin level and hemoglobin level had no relation(P＞ 0.05 ). Multivariate analysis revealed that renal dysfunction at the time of diagnosis was a significant and independent prognostic factor for survival (P ＜0.05). Conclusions Renal dysfunction at the time of diagnosis is the best predictor of survival. The presence of amyloidosis in organs other than the kidney, such as advanced cardiac amyloidosis, predicts a poor survival. Key words: Prognosis; Renal amyloidosis; Immunoglobulin light-chain; Survival analysis

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中国医师进修杂志

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期刊介绍： Chinese Journal of Postgraduates of Medicine (founded in 1978, monthly) is one of the series of journals of the Chinese Medical Association (CMA) under the supervision of the National Health Commission of the People's Republic of China, sponsored by the Chinese Medical Association (CMA) and Dalian Institute of Theoretical Medicine (DITM). It is a core journal of Chinese science and technology, a statistical source journal of Chinese scientific and technical papers, and a statistical source journal of China Academic Journal Impact Factor Annual Report. The journal adheres to the purpose of using continuing medical education and post training, highlights new theories, new knowledge, new methods and new technologies, and focuses on the combination of theory and practice, and the combination of popularisation and improvement. Main columns: Special tutorials, expert forums, clinical treatises, clinical experience, reviews and lectures, continuing medical education, teaching and research, advanced training forum, drugs and clinics, overview of progress, introduction of new technologies, imaging and clinics, lessons learnt, discussions and evaluations, case reports, discussions of clinical cases, conference proceedings, and domestic and international academic developments. The main target readers are clinical medical personnel in internal medicine, surgery, gynaecology, paediatrics and ophthalmology.