摘要
老年男性,慢性起病,临床表现为肾病综合征,少量镜下血尿,肾功能正常,高血压,轻度贫血,免疫固定电泳见κ型IgG单克隆免疫球蛋白条带.国家肾脏疾病临床医学研究中心首次肾活检示肾小球膜增生样病变,免疫荧光肾小球IgG3、κ轻链沉积为主,电镜下肾小球系膜区、基膜内皮下少量无特殊结构的电子致密物沉积;同时肾小球袢腔内CD68^+巨噬细胞胞质内见嗜伊红、嗜复红颗粒,为增生性肾小球肾炎伴单克隆IgG沉积(PGNMID)伴肾小球巨噬细胞及系膜细胞内异常内含物.经硼替佐米联合地塞米松化疗后临床部分缓解,8个月后重复肾活检示肾小球细胞增生较前减轻,IgG消失,κ轻链明显减弱,胞质内内含物几近消失.
A 68-year-old man presented as nephrotic syndrome,hematuria,hypertension,anemia,and monoclonal IgG-κ in serum immunofixation electrophoresis(IFE).Renal biopsy revealed membranoproliferative glomerulonephritis(MPGN),with IgG3-κ staining in glomeruli and non-organized electron-dense deposition in the mesangium and subendothelial area.In addition,CD68+ macrophages with eosinophilic droplets or globules in cytoplasm within segmental capillary loops were κ light chain positive on immunofluorescence.Electron microscopy showed intracytoplasmic crystalloid inclusions within macrophages and mesangial cells.The final diagnosis was proliferative glomerulonephritis with monoclonal immunoglobulin deposits(PGNMID) with glomerular intracellular crystalloid inclusions.He was treated with bortezomib and dexamethasone,and achieved a partial response.After 8 months,arepeated renal biopsy demonstrated that active glomerular proliferation reversed.And IgG,κ light chain and crystalloid inclusions were almost disappeared.
作者
梁少姗
曾彩虹
LIANG Shaoshan;ZENG Caihong(National Clinical Research Center of Kidney Diseases,Jinling Hospital,Nanjing University School of Medicine,Nanjing 210016,China)
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
北大核心
2020年第3期291-295,共5页
Chinese Journal of Nephrology,Dialysis & Transplantation
关键词
增生性肾小球肾炎伴单克隆IgG沉积
巨噬细胞
结晶
内含物
proliferative glomerulonephritis with monoclonal immunoglobulin deposits
macrophage
crystal
inclusion
