伴单克隆IgG1沉积的增生性肾小球肾炎

doi:10.3969/j.issn.1006-298X.2018.05.020

肾脏病与透析肾移植杂志 ›› 2018, Vol. 27 ›› Issue (5): 493-497.DOI: 10.3969/j.issn.1006-298X.2018.05.020

伴单克隆IgG1沉积的增生性肾小球肾炎

出版日期:2018-10-28 发布日期:2018-11-14

Proliferative glomerulonephritis with monoclonal IgG1 deposits

Online:2018-10-28 Published:2018-11-14

摘要/Abstract

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IgG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），IgG1++、λ轻链+颗粒状沉积于系膜区及血管袢，IgG2、IgG3、IgG4、κ轻链阴性；诊断为伴单克隆IgG1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。

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

Abstract:

61yearold female was admitted to the hospital with proteinuria,microscopic hematuria,anemia,hypocomplementemia for 10 months.Serum test disclosed monoclonal IgGλ band and elevated λ free light chain(λ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 λ light chain (+) in the mesagium and capoillary walls,while IgG2,IgG3,IgG4 and κ 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 κ/λ ratio normalized,hypocomplementemia improved.

Key words: monoclonal gammapathy, membranoproliferative glomerulonephritis, thalidomide, dexamethasone

李娟，周伟，程震，等. 伴单克隆IgG1沉积的增生性肾小球肾炎[J]. 肾脏病与透析肾移植杂志, 2018, 27(5): 493-497.

LI Juan,ZHOU Wei,CHENG Zhen,et al. Proliferative glomerulonephritis with monoclonal IgG1 deposits[J]. Chinese Journal of Nephrology, Dialysis & Transplantation, 2018, 27(5): 493-497.

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ISSN 1006-298X CN 32-1425/R

伴单克隆IgG1沉积的增生性肾小球肾炎

Proliferative glomerulonephritis with monoclonal IgG1 deposits

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