达雷妥尤单抗联合环磷酰胺治疗伴单克隆免疫球蛋白沉积的增生性肾小球肾炎1例  

Daratumumab combined with cyclophosphamide in the treatment of proliferative glomerulonephritis with monoclonal immunoglobulin deposits:a case report

作　　者：邱锋平 陈亮亮 蔡方豪 周芹 陈江华 韩飞 Qiu Fengping;Chen Liangliang;Cai Fanghao;Zhou Qin;Chen Jianghua;Han Fei(Kidney Disease Center,the First Affiliated Hospital,College of Medicine,Zhejiang University/Institute of Nephrology,Zhejiang University/Key Laboratory of Kidney Disease Prevention and Control Technology,Hangzhou 310003,China;Department of Nephrology,the First People's Hospital of Huzhou,Huzhou 313000,China)

机构地区：[1]浙江大学医学院附属第一医院肾脏病中心,浙江大学肾脏病研究所,浙江省肾脏病防治技术研究重点实验室,杭州310003 [2]湖州市第一人民医院肾内科,湖州313000

出　　处：《中华肾脏病杂志》2024年第8期663-667,共5页Chinese Journal of Nephrology

摘　　要：该文报道1例少见的伴单克隆免疫球蛋白沉积的增生性肾小球肾炎病例。患者中年女性,临床表现为肾病综合征、镜下血尿、肾功能不全、高血糖及双眼糖尿病视网膜病变,血、尿免疫固定电泳均未见单克隆条带。肾活检病理光镜下为膜增生性肾小球肾炎,免疫荧光IgG亚型中只有IgG3沿着毛细血管袢呈花瓣状沉积;电镜下肾小球基底膜节段性增厚,内皮下、系膜区可见电子致密物沉积,未见特殊有形结构,诊断为伴单克隆免疫球蛋白沉积的增生性肾小球肾炎合并糖尿病肾病。患者在接受达雷妥尤单抗联合环磷酰胺针序贯治疗6个月后,肾病综合征部分缓解,肾功能恢复正常,贫血明显改善,随访24个月,无严重不良反应发生。This article reports a rare case of proliferative glomerulonephritis with monoclonal immunoglobulin deposits.The patient,a middle-aged woman,exhibited clinical manifestations including nephrotic syndrome,microscopic hematuria,renal insufficiency,hyperglycemia,and bilateral diabetic retinopathy.Notably,monoclonal bands were absent in both blood and urine immunofixation electrophoresis.Renal biopsy revealed membranoproliferative glomerulonephritis,with immunofluorescence revealing exclusive petal-like deposition of IgG3 subtype along the capillary loop.Electron microscopy demonstrated segmental thickening of the glomerular basement membrane,along with electron dense deposits in the subendothelial and mesangial areas,lacking discernible substructure.The diagnosis comprised proliferative glomerulonephritis with monoclonal immunoglobulin deposition concurrent with diabetic nephropathy.Subsequent sequential treatment with daratumumab and cyclophosphamide over 6 months led to partial remission of nephrotic syndrome,normalization of renal function,and significant improvement of anemia.During the 24-month follow-up period,no serious adverse reactions occurred.

关 键 词：副蛋白血症 肾小球肾炎 膜增生性 环磷酰胺 达雷妥尤单抗 单克隆免疫球蛋白 增生性肾小球肾炎 

分 类 号：R692.31[医药卫生—泌尿科学]