摘要
目的 探讨轻链肾病 (lightchainnephropathy ,LCN)的病理学特点及其诊断方法。方法对7例LCN进行了系统的光镜、免疫荧光及透射电镜检查 ,并进行轻链蛋白 (κ、λ)的免疫荧光和免疫电镜标记。结果 7例的光镜表现不一 ,3例系膜结节状硬化性肾小球病 ,1例系膜轻至中度增生 ;其余3例为管型肾病 ,肾小球病变轻微。免疫荧光检查 :7例均可见单一品种的轻链蛋白在肾小球系膜区团块状 ,及肾小球基底膜、肾小管基底膜和小血管壁呈线性沉积。透射电镜检查可见肾小球系膜区和基底膜内侧、肾小管基底膜外侧及小血管壁的颗粒状电子致密物沉积。免疫电镜标记显示 ,肾小球基底膜内侧和系膜区、肾小管基底膜外侧及小血管壁的颗粒状沉积物均可被单一品种轻链蛋白 (5例κ,2例λ)标记。结论 典型的LCN呈系膜结节状硬化性肾小球病 ,电镜观察到LCN的特征性超微结构改变具有诊断意义 ,结合轻链蛋白 (κ、λ)的免疫荧光或免疫电镜检查可进一步确诊 ,尤其对光镜病变不明显的LCN更有意义。
Objective To investigate the pathologic features and diagnostic algorithm of light chain nephropathy (LCN). Methods Seven cases of LCN were studied by light microscopy, electron microscopy and immunolabeling of light chains (κ,λ) by immunofluorescence and immunoelectron microscopy. Results The histopathology of 7 cases by light microscopy was variable, with 3 cases showing nodular glomerulosclerosis, 1 case showing mild to moderate mesangial proliferation, and 3 cases showing cast nephropathy with minimal glomerular change. Immunofluorescence study revealed positive staining of a single type of light chain in mesangium (nodular pattern) or along glomerular basement membrane (linear), along tubular basement membrane and around arteriolar walls in all the 7 cases. Ultrastructurally, electron-dense granular deposits were identified in mesangium, subendothelial aspect of glomerular basement membrane, outer aspect of tubular basement membrane and arteriolar walls. Immunogold labeling of light chains showed distinct labeling of a single type light chain in the granular electron-dense materials (5 cases being κ-positive and 2 being λ-positive). Conclusions LCN typically shows nodular glomerulosclerosis. The ultrastructural change is characteristic and important for diagnosis. Immunolabeling of light chains by immunofluorescence and immunoelectron microscopy carries further diagnostic value, especially in cases with minimal light microscopic change.
出处
《中华病理学杂志》
CAS
CSCD
北大核心
2003年第6期506-510,共5页
Chinese Journal of Pathology
