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一名恶性淋巴瘤患者出现广泛的肾小球内单克隆IgM-κ血栓。

Extensive intraglomerular thrombi of monoclonal IgM-kappa in a patient with malignant lymphoma.

作者信息

Oyama Y, Komatsuda A, Ohtani H, Imai H, Kitabayashi A, Yamaguchi A, Nimura T, Miura A B, Nakamoto Y

机构信息

Third Department of Internal Medicine, Akita University School of Medicine, Japan.

出版信息

Am J Kidney Dis. 2000 Mar;35(3):E11. doi: 10.1016/s0272-6386(00)70223-x.

Abstract

We describe an 80-year-old man who developed malignant lymphoma (ML) complicated by extensive intraglomerular thrombi of immunoglobulin M (IgM)-kappa monoclonal immunoglobulin. The clinical picture was characterized by nephrotic syndrome and systemic lymphadenopathy. Laboratory examination showed mild anemia and a small amount of monoclonal IgM-kappa in the blood. The histopathologic findings and surface immunoglobulin analysis of the lymph node biopsy specimen were consistent with CD5-positive diffuse large B-cell (type, IgM-kappa) lymphoma. The subsequent renal biopsy showed a massive deposition of amorphous material in the glomerular capillary lumens, subendothelial areas, and mesangium. Nodular glomerulosclerosis was not found. An immunofluorescent study showed that the deposits consisted of IgM-kappa monoclonal immunoglobulin. Ultrastructurally, the deposits were composed of granular electron-dense material. Chemotherapy was effective for both the ML and nephrotic syndrome, and the patient's urine analysis results returned to normal. The histopathologic manifestations of this case are rare, and the pathogenesis of these glomerular lesions was obviously associated with ML.

摘要

我们描述了一名80岁男性,他患有恶性淋巴瘤(ML),并发免疫球蛋白M(IgM)-κ单克隆免疫球蛋白广泛的肾小球内血栓形成。临床表现为肾病综合征和全身淋巴结病。实验室检查显示轻度贫血,血液中有少量单克隆IgM-κ。淋巴结活检标本的组织病理学发现和表面免疫球蛋白分析与CD5阳性弥漫性大B细胞(类型,IgM-κ)淋巴瘤一致。随后的肾活检显示在肾小球毛细血管腔、内皮下区域和系膜中有大量无定形物质沉积。未发现结节性肾小球硬化。免疫荧光研究表明,沉积物由IgM-κ单克隆免疫球蛋白组成。超微结构上,沉积物由颗粒状电子致密物质组成。化疗对ML和肾病综合征均有效,患者的尿液分析结果恢复正常。该病例的组织病理学表现罕见,这些肾小球病变的发病机制明显与ML有关。

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