Wang Suxia, Zou Wanzhong, Wang Mei, Xie Yanling, Chai Lijun, Tang Xiuying
Lab of Electron Microscopy, Peking University First Hospital, Beijing 100034, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2003 Dec 18;35(6):576-80.
To investigate the role of electron microscopy (EM) and immunoelectron microscopy (IEM) in the diagnosis of light chain deposition diseases (LCDD) and primary amyloidosis (AL) of the kidney. METHODS Renal biopsy specimens collected in our hospital from Jan. 1994 to Feb. 2002 were studied by EM, and ultrastructural immunogold labeling for light chains (kappa, Lambda) were made on the cases suggested of LCDD and early stage AL in the preliminary diagnosis.
Six cases were diagnosed as LCDD, and punctate, granular, electron-dense material was identified in the outside of tubular basement membrane (TBM), inner layer of glomerular basement membrane (GBM) and vascular walls by EM. Two of which showed interrupted segmental localization of granular material in subendothelial areas of GBM. The granular material was labeled by monotypical light chains (4 cases of kappa chain, 2 cases of Lambda chain). Thirty-six cases were diagnosed as AL. EM revealed fibrillary material distributed in glomerular mesangium, GBM and vascular walls; 15 of them showed segmental distribution of fibrils in mesangium and/or GBM, and were further studied by IEM labeling for light chains. The fibrillary material was labeled by monotypical light chains (4 cases of kappa chain, 11 cases of Lambda chain). CONCLUSION The diagnosis of renal LCDD and early stage of AL depended on EM, IEM is a unique diagnostic tool in early stage of LCDD and AL.
探讨电子显微镜(EM)及免疫电子显微镜(IEM)在肾轻链沉积病(LCDD)和原发性淀粉样变性(AL)诊断中的作用。方法 对我院1994年1月至2002年2月收集的肾活检标本进行EM检查,并对初步诊断提示为LCDD和早期AL的病例进行轻链(κ、λ)超微结构免疫金标记。
6例诊断为LCDD,EM检查在肾小管基底膜(TBM)外侧、肾小球基底膜(GBM)内层及血管壁发现点状、颗粒状电子致密物质。其中2例GBM内皮下区域颗粒物质呈节段性间断分布。颗粒物质被单克隆轻链标记(κ链4例,λ链2例)。36例诊断为AL。EM显示纤维状物质分布于肾小球系膜、GBM及血管壁;其中15例在系膜和/或GBM呈节段性纤维分布,并进一步行轻链IEM标记研究。纤维状物质被单克隆轻链标记(κ链4例,λ链11例)。结论 肾LCDD及AL早期诊断依赖EM,IEM是LCDD和AL早期独特的诊断工具。