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华氏巨球蛋白血症中免疫介导的肾脏疾病。

Immunologically-mediated renal disease in Waldenström's macroglobulinemia.

作者信息

Martelo O J, Schultz D R, Pardo V, Perez-stable E

出版信息

Am J Med. 1975 Apr;58(4):567-75. doi: 10.1016/0002-9343(75)90133-3.

Abstract

A patient with Walderström macroglobulinemia associated with nephrotic syndrome is described. Serum cryoglobulin and rheumatoid factor were absent. Intramembranous electron-dense deposits were demonstrated in kidney biopsy material by electron microscopy. Deposits of immunoglobulin G (IgG), M (IgM) and the third component of complement (C3) were identified in kidney biopsy tissue by immunofluorescent staining methods. The serum immunoglobulins were characterized by chromatographic and immunochemical methods and showed a monocional IgM-K, IgG-K and gamma-chain piece of undefined structure. Free K- and gamma-chains were found in the urine. The IgM was not complexed to the IgG or vice versa, but the IgG was in an affregated form. Although it is not known which immunoglobulin initiated the tissue injury, IgG, IgM and complement deposits probably contributed to the renal dysfunction. The nephrotic syndrome diminished after treatemnt with chlorambucin and corticosteroids.

摘要

本文描述了一名患有与肾病综合征相关的华氏巨球蛋白血症患者。血清冷球蛋白和类风湿因子均未检出。通过电子显微镜在肾脏活检材料中发现了膜内电子致密沉积物。通过免疫荧光染色方法在肾脏活检组织中鉴定出免疫球蛋白G(IgG)、M(IgM)和补体第三成分(C3)的沉积物。采用色谱法和免疫化学方法对血清免疫球蛋白进行了表征,结果显示为单克隆IgM-K、IgG-K和结构未明的γ链片段。尿液中发现了游离的K链和γ链。IgM未与IgG形成复合物,反之亦然,但IgG呈聚集形式。虽然尚不清楚哪种免疫球蛋白引发了组织损伤,但IgG、IgM和补体沉积物可能导致了肾功能障碍。使用苯丁酸氮芥和皮质类固醇治疗后,肾病综合征有所减轻。

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