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
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。
更多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.
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