伴单克隆IgG1沉积的增生性肾小球肾炎  

Proliferative glomerulonephritis with monoclonal IgG1 deposits

作　　者：李娟 周伟 程震 梁少姗 刘志红 LI Juan;ZHOU Wei;CHENG Zhen;LIANG Shaoshan;LIU Zhihong(National Clinical Research Center of Kidney Diseases,Jinling Hospital,Nanjing University School of Medicine,Nanjing 210016,China)

机构地区：[1]南京总医院国家肾脏疾病临床医学研究中心全军肾脏病研究所,南京210016

出　　处：《肾脏病与透析肾移植杂志》2018年第5期493-497,492,共6页Chinese Journal of Nephrology,Dialysis & Transplantation

摘　　要：61岁女性,病程10个月,临床表现尿检异常、低补体血症伴贫血、血游离轻链比值异常,免疫固定电泳见Ig G-λ单克隆免疫球蛋白条带,肾脏体积增大,骨髓浆细胞7. 5%、未见原始和幼稚浆细胞,肾活检示膜增生性肾小球肾炎(结节样病变),Ig G1++、λ轻链+颗粒状沉积于系膜区及血管袢,Ig G2、Ig G3、Ig G4、κ轻链阴性;诊断为伴单克隆Ig G1沉积的增生性肾小球肾炎。给予沙利度胺联合地塞米松治疗1年,蛋白尿完全缓解,补体上升,血游离轻链比值恢复正常,血清M蛋白转阴。61-year-old female was admitted to the hospital with proteinuria, microscopic hematuria, anemia, hypocomplementemia for 10 months.Serum test disclosed monoclonal IgG-h band and elevated h free light chain（ h-FLC ）. Ultrasonography showed bilateral kidney enlargement, bone marrow cytology disclosed 7.5% of plasmacyte with normal morphology. A kidney biopsy was carried out. On light microscopy, the main manifestation is membranoproliferative glomerulonephritis with nodular lesions ,IF showed granular staining of IgG1 （＋＋） and h light chain （＋） in the mesagium and capoillary walls, while IgG2, IgG3, IgG4 and K light chain were negative. The final diagnosis was proliferative glomerulonephritis with monoclonal IgG1 deposits,the patient was treated with thalidomide and dexamethasone for 1 year, On last follow up ,the proteinuria and M protein disappeared, serum v,/h ratio normalized, hypocomplementemia improved.

关 键 词：单克隆免疫球蛋白 肾小球膜增生性病变 沙利度胺 地塞米松 

分 类 号：R692.31[医药卫生—泌尿科学]