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具有肾意义的单克隆丙种球蛋白病(MGRS)中的晶体样肾小球病

Crystalloid glomerulopathy in monoclonal gammopathy of renal significance (MGRS).

作者信息

Vankalakunti Mahesha, Bonu Ravishankar, Shetty Shilpa, Siddini Vishwanath, Babu Kishore, Ballal Sudarshan H

机构信息

Department of Pathology , Manipal Hospital , Bangalore , India.

Department of Nephrology , Manipal Hospital , Bangalore , India.

出版信息

Clin Kidney J. 2014 Jun;7(3):296-8. doi: 10.1093/ckj/sfu025. Epub 2014 Mar 18.

Abstract

We report a case of monoclonal gammopathy of renal significance in a 63-year-old man who presented with nephrotic-range proteinuria and renal insufficiency. The kidney biopsy showed a membranoproliferative glomerulonephritis pattern with extensive crystalloid deposits in the glomerular capillary endothelial cells and very few in the tubular epithelial cells. The immunoperoxidase staining showed kappa light chain restriction. Subsequently, the bone marrow showed 6% plasma cells which confirmed the diagnosis of monoclonal gammopathy of renal significance. He responded well to bortezomib treatment with resolution of the nephrotic syndrome and normalization of renal function after 7 months.

摘要

我们报告了一例具有肾脏意义的单克隆丙种球蛋白病,患者为一名63岁男性,表现为肾病范围蛋白尿和肾功能不全。肾活检显示膜增生性肾小球肾炎模式,肾小球毛细血管内皮细胞中有广泛的晶体沉积,肾小管上皮细胞中则很少。免疫过氧化物酶染色显示κ轻链限制。随后,骨髓检查发现6%的浆细胞,这证实了具有肾脏意义的单克隆丙种球蛋白病的诊断。他对硼替佐米治疗反应良好,7个月后肾病综合征消退,肾功能恢复正常。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/372c/4377747/7691ba82d53e/sfu02501.jpg

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