Crystalloid glomerulopathy in monoclonal gammopathy of renal significance (MGRS).
Crystalloid glomerulopathy in monoclonal gammopathy of renal significance (MGRS).
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DOI：
10.1093/ckj/sfu025
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发表时间：
2014-06
影响因子：
4.6
通讯作者：
Ballal SH
中科院分区：
文献类型：
--
作者：
Vankalakunti M;Bonu R;Shetty S;Siddini V;Babu K;Ballal SH
我们报告一例单克隆丙种球蛋白病的肾脏意义的63岁男子谁提出了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大量晶体沉积，肾小管上皮细胞中很少。免疫过氧化物酶染色显示κ轻链限制性。随后，骨髓显示6%的浆细胞，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，肾病综合征消退，7个月后肾功能正常化。
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.