单克隆免疫球蛋白轻链相关性肾病的超微结构及免疫电镜研究  

Ultrastructural and immunoelectron microscopic study on monoclonal light chain-related renal diseases

作　　者：王素霞[1] 邹万忠[2] 张烨[1] 谢燕玲[1] 王书合[1] 柴立军[1] 汤秀英[1] 

机构地区：[1]北京大学第一医院电镜室,北京100034 [2]北京大学基础医学院病理系,北京100083

出　　处：《电子显微学报》2004年第1期9-15,共7页Journal of Chinese Electron Microscopy Society

摘　　要：目的:探讨电镜及免疫电镜方法在单克隆免疫球蛋白轻链相关性肾病包括轻链沉积病(lightchaindepositiondisease,LCDD)和轻链型淀粉样变(amyloidosis,AL)诊断中的作用。方法:对我院1994 1～2002 12间接受的肾活检病例进行系统的电镜观察,对疑为LCDD及早期AL的病例进行轻链(κ、λ)的免疫电镜标记。结果:7例符合LCDD的诊断,45例符合AL的诊断。LCDD肾活检组织电镜下可见肾小管基底膜(TBM)外侧、肾小球基底膜(GBM)内侧、鲍曼囊外侧及小血管壁基底膜外侧的点状、颗粒状电子致密物质沉积,其中2例早期病例表现颗粒状物质节段性分布;免疫电镜标记,颗粒状物质被单种轻链标记,其中,κ轻链5例,λ轻链2例。AL肾活检组织电镜下可见肾小球系膜区、GBM及小动脉壁基底膜上的细纤维物质呈团片状或束状分布,其中15例早期病例表现系膜区或 及GBM的细纤维呈节段性分布;15例早期AL的免疫电镜标记结果,细纤维被单种轻链标记,其中,κ轻链4例,λ轻链11例。结论:肾脏LCDD及早期AL的诊断需依赖于电镜检查,免疫电镜检查对肾脏早期LCDD及早期AL的确诊具有不可替代的作用。Objective:To investigate the role of electron microscopy (EM) and immunoelectron microscopy (IEM) in the diagnosis of monoclonal light chain-related renal diseases including light chain deposition diseases(LCDD) and primary amyloidosis(AL). Method: Renal biopsy specimens collected in our hospital from Jan. 1994 to Dec. 2002 were studied by EM, ultrastructural immunogold labeling for light chains (κ、λ) were made on the cases suggested of LCDD and early stage AL in the preliminary diagnosis. Results: Seven cases were diagnosed as LCDD, punctate, granular, electron-dense material was identified in the outside of tubular basement membrane (TBM), inner layer of glomerular basement membrane (GBM) and outside of vascular basement membrane by EM. Two of them showed interrupted segmental localization of granular material in subendothelial areas of GBM. The granular material was labeled by monotypical light chains (5 cases of κ chain, 2 cases of λ chain). 45 cases were diagnosed as AL, EM revealed fibrillary material distributed in glomerular mesangium, GBM and arteriolar basement membrane; 15 of them showed segmental distribution of microfibrils in mesangium and/or GBM, and were further studied by IEM labeling for light chains. The microfibrils were labeled by monotypical light chains (4 cases of κ chain, 11 cases of λ chain). Conclusion: The diagnosis of renal LCDD and early stage of AL depended on EM. IEM was a unique diagnostic tool in early stage of LCDD and AL.

关 键 词：肾脏 轻链沉积病 淀粉样变 电子显微镜 免疫金标记 免疫电镜法 

分 类 号：R692.9[医药卫生—泌尿科学]