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与结节性肾小球硬化相关的非分泌性骨髓瘤。

Nonsecretory myeloma associated with nodular glomerulosclerosis.

作者信息

Sølling K, Sølling J, Jacobsen N O, Thomsen O F

出版信息

Acta Med Scand. 1980;207(1-2):137-43. doi: 10.1111/j.0954-6820.1980.tb09693.x.

Abstract

A patient with nonsecretory multiple myeloma in associated with nodular glomerulosclerosis is reported. The clinical course was characterized by rapidly progressing renal insufficiency terminating in uremia within three months. Histological investigation of the kidney revealed extensive nodular formations in the mesangial areas consisting of basement membrane-like material, in places with content of fibrils. Immunofluorescence demonstrated deposition of kappa chains. Amyloid was not present. Routine methods for investigation of serum and urine showed severe hypogammaglobulinemia without M-components. Using a sensitive radioimmunoassay for free light chains, an abnormal non-dissociable kappa chain polymer with a molecular weight of 55 000 was found in serum and urine. The findings support the hypothesis that mesangial accumulation of paraproteins induce an increased synthesis of basement membrane material leading to formation of the nodules. Abnormal polymerization of kappa chains might provoke the formation of the glomerular nodules.

摘要

报告了一例非分泌型多发性骨髓瘤合并结节性肾小球硬化症的患者。临床病程的特点是肾功能迅速恶化,在三个月内发展为尿毒症。肾脏组织学检查显示,系膜区有广泛的结节形成,由基底膜样物质组成,部分区域含有纤维。免疫荧光显示κ链沉积。未发现淀粉样物质。血清和尿液的常规检查方法显示严重低丙种球蛋白血症,无M成分。使用敏感的游离轻链放射免疫分析法,在血清和尿液中发现了一种分子量为55000的异常非解离κ链聚合物。这些发现支持以下假说:副蛋白在系膜中的积聚导致基底膜物质合成增加,从而形成结节。κ链的异常聚合可能引发肾小球结节的形成。

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