肾脏轻链-重链沉积病一例并文献复习

目的 报告1例肾脏轻链-重链沉积病（IgGκ轻链-γ1重链）.方法 分析临床表现,血清免疫固定电泳,血、尿中轻、重链异常,骨髓活检和肾活检病理资料.结果 临床表现大量蛋白尿,镜下血尿,高血压,贫血,肾功能正常.免疫固定电泳见κ型IgG条带,伴血、尿液中轻链升高.骨髓浆细胞4％.肾活检肾小球呈“结节样病变”,刚果红染色阴性.免疫荧光κ轻链和IgG1（y1重链单抗）沿肾小球基膜（GBM）和肾小管基膜（TBM）线样沉积.电镜见GBM内侧及TBM外侧高电子致密物沉积.结论 肾脏轻-重链沉积病（IgGκ轻链-γ1重链）的诊断,应临床结合病理,特别是进行免疫病理检查.

One case report and analysis of patient with renal immunoglobulin light and heavy chain deposited disease

Objective To report 1 case with renal immunoglobulin light and heavy chain deposited disease （IgG-κ light chain and γ1 heavy chain）.Methods The clinical manifestations,serum immunofixation electrophoresis,light and heavy chain abnormalities of blood and urine,bone marrow biopsy and renal biopsy data laboratory data were recorded and analyzed.Results A 63-year-old woman presented with massive proteinuria,microscopic hematuria,hypertension,anemia and serum IgG-κ light chain.Bone marrow aspirate revealed 4% plasma cells.Renal biopsy revealed nodular glomerulopathy with congo red staining-negative.Immunofluorescence showed that κ light chain and IgG1 （γ1 heavy chain） were deposited along the glomerular basement membranes （GBM） and tubular basement membranes （TBM）.Electron-microscopy revealed electrondense deposits that delineate the outer aspect of TBM and endothelial aspect of GBM.Conclusion The diagnosis of renal immunoglobulin light and heavy chain deposited disease （IgG-κ light chain and γ1 heavy chain） should be addressed combine with clinical and is pathology,especially with immune pathological examination.