Oe Yuji, Joh Kensuke, Sato Mitsuhiro, Taguma Yoshio, Onishi Yasushi, Nakayama Keisuke, Sato Toshinobu
Department of Nephrology, Sendai Shakai Hoken Hospital, Sendai, Japan.
Division of Nephrology, Endocrinology and Vascular Medicine, Graduate School of Medicine, Tohoku University, 1-1 Seiryo-cho, Sendai, 980-8574, Japan.
CEN Case Rep. 2013 Nov;2(2):222-227. doi: 10.1007/s13730-013-0068-z. Epub 2013 Mar 1.
A 48-year-old man with chronic lymphocytic leukemia presented with nephrotic syndrome, hematuria, and mild deterioration of renal function. Further analysis using serum immunofixation electrophoresis detected monoclonal immunoglobulin (Ig) M-κ and IgG-κ M-protein. Testing for cryoglobulin in serum was negative. Light microscopy of a renal biopsy specimen showed membranoproliferative glomerulonephritis features with marked mononuclear cell infiltration in the interstitium. On immunofluorescence study, the deposition of IgM heavy chain was predominantly observed with the same distribution of κ light chain, whereas no λ light chain was found. Electron microscopy revealed fine granular deposits in the mesangial, subendothelial, and subepithelial areas, mimicking those observed in the immune complex-mediated glomerulonephritis. These pathological findings were consistent with recently described cases of proliferative glomerulonephritis with monoclonal IgG deposits. Thus, monoclonal IgM deposition can also cause proliferative glomerulonephritis.
一名48岁的慢性淋巴细胞白血病男性患者出现肾病综合征、血尿及肾功能轻度恶化。血清免疫固定电泳进一步分析检测到单克隆免疫球蛋白(Ig)M-κ和IgG-κ M蛋白。血清冷球蛋白检测为阴性。肾活检标本的光镜检查显示膜增生性肾小球肾炎特征,间质有明显单核细胞浸润。免疫荧光研究显示,主要观察到IgM重链沉积,κ轻链分布相同,未发现λ轻链。电子显微镜显示系膜、内皮下和上皮下区域有细颗粒状沉积物,类似于免疫复合物介导的肾小球肾炎中观察到的沉积物。这些病理结果与最近描述的伴有单克隆IgG沉积的增生性肾小球肾炎病例一致。因此,单克隆IgM沉积也可导致增生性肾小球肾炎。