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无华氏巨球蛋白血症的单克隆IgM沉积增殖性肾小球肾炎:病例报告及文献复习

Proliferative glomerulonephritis with monoclonal IgM deposits without Waldenström's macroglobulinemia: case report and review of the literature.

作者信息

Yahata Mayumi, Nakaya Izaya, Takahashi Satoko, Sakuma Tsutomu, Sato Hiroshi, Soma Jun

机构信息

Departments of Nephrology, Iwate Prefectural Central Hospital Morioka, Iwate, Japan.

出版信息

Clin Nephrol. 2012 Mar;77(3):254-60. doi: 10.5414/cn107230.

Abstract

A 38-year-old man was presented with nephrotic syndrome associated with hematuria and mild hypocomplementemia. Renal biopsy revealed lobular mesangial proliferation, thickened capillary walls, and intraluminal protein thrombi. Immunofluorescence showed marked and mild depositions of immunoglobulin (Ig) M heavy chains and complement C3, respectively, in a peripheral lobular pattern. On light chain staining, only kappa (κ) light and IgM heavy chains showed a similar pattern. Electron microscopy showed fine granular electrondense deposits in subendothelial areas and numerous globular deposits (varying size and density) in glomerular capillary lumens. Serum levels of Ig κ, but not of free κ, light chains were significantly increased. Bone marrow aspiration revealed a normocellular marrow. Waldenström's macroglobulinemia and cryoglobulinemia were ruled out. Clinically, steroid therapy was ineffective and proteinuria persisted. This report demonstrates that glomerular deposition of monoclonal IgM-κ can produce membranoproliferative- like changes in the glomeruli. This condition may be recognized as proliferative glomerulonephritis with monoclonal IgM deposits similar to the recently recognized proliferative glomerulonephritis with monoclonal IgG deposits.

摘要

一名38岁男性患者出现肾病综合征,伴有血尿和轻度补体低下。肾活检显示小叶性系膜增生、毛细血管壁增厚和管腔内蛋白血栓形成。免疫荧光显示免疫球蛋白(Ig)M重链和补体C3分别以周边小叶模式显著和轻度沉积。轻链染色显示,仅κ轻链和IgM重链呈现相似模式。电子显微镜检查显示内皮下区域有细小颗粒状电子致密沉积物,肾小球毛细血管腔内有大量球状沉积物(大小和密度各异)。血清Ig κ轻链水平显著升高,但游离κ轻链水平未升高。骨髓穿刺显示骨髓细胞正常。排除了华氏巨球蛋白血症和冷球蛋白血症。临床上,类固醇治疗无效,蛋白尿持续存在。本报告表明,单克隆IgM-κ在肾小球的沉积可导致肾小球出现膜增生样改变。这种情况可能被认为与最近认识的伴有单克隆IgG沉积的增生性肾小球肾炎相似,是伴有单克隆IgM沉积的增生性肾小球肾炎。

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