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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 and immunoelectron microscopy in the diagnosis of monoclonal immunoglobulin light chain-related nephropathy, including light chain deposition disease (LCDD) and light chain amyloidosis (AL). Methods: 12 cases of renal biopsy accepted by our hospital from 1994 to 2002 were systematically observed by electron microscopy, and immunostaining with light chain (κ, λ) was performed on suspected cases of LCDD and early AL. Results: Seven cases were diagnosed with LCDD and 45 cases were diagnosed with AL. LCDD renal biopsy under the electron microscope can be seen outside the tubular basement membrane (TBM), glomerular basement membrane (GBM) medial, Bowman’s capsule and small vessel wall outside the basal membrane of dot-like, granular electron dense material deposition, of which 2 Cases of early cases showed segmental distribution of granular material; immunoelectron microscopy, granular material was a single light chain markers, including kappa light chain in 5 cases, λ light chain in 2 cases. AL renal biopsy under electron microscopy showed glomerular mesangial area, GBM and arteriolar basilar membrane fibrillar material was clustered or bundle distribution, of which 15 cases of early cases of mesangial area or GBM fine fibers Showed segmental distribution. In the early stage of 15 cases, the results of immunoelectron microscopy showed that the fine fibers were labeled with a single light chain, including 4 cases of kappa light chain and 11 cases of lambda light chain. Conclusion: The diagnosis of renal LCDD and early AL needs to rely on electron microscopy. Immunoelectron microscopy has an irreplaceable effect on the diagnosis of early LCDD and early AL in the kidney.