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肾功能不全骨髓瘤患者血清中的聚合本-周蛋白

Polymeric Bence Jones proteins in serum in myeloma patients with renal insufficiency.

作者信息

Sølling K, Sølling J, Lanng Nielsen J

出版信息

Acta Med Scand. 1984;216(5):495-502. doi: 10.1111/j.0954-6820.1984.tb05037.x.

Abstract

The polymeric forms of Bence Jones protein (BJ) in serum and the influence on renal function were investigated in 59 patients with multiple myeloma and 2 with Waldenström's macroglobulinemia. Eight of 35 patients with kappa and 26 with lambda type BJ protein had decreased renal function. The investigation showed a considerable variation between patients in serum concentrations of tetrameric (T), dimeric (D) and monomeric (M) forms of BJ protein. A significantly higher fraction of D forms of BJ protein resulting in a high D/M ratio was found in the myeloma patients compared with polyclonal light chains in 10 normal controls. The renal function in multiple myeloma was, however, not correlated to the D/M ratio or to the BJ protein tetramers. This suggests that the nephrotoxicity of BJ proteins is not associated with polymerization of BJ proteins in serum. BJ proteins of aberrant size were found in 3 patients with rapidly decreasing renal function. Histological investigation in 2 of these patients revealed nodular glomerulosclerosis.

摘要

对59例多发性骨髓瘤患者和2例华氏巨球蛋白血症患者的血清中本-周蛋白(BJ)的聚合形式及其对肾功能的影响进行了研究。35例κ型和26例λ型BJ蛋白患者中有8例肾功能下降。研究表明,患者血清中BJ蛋白的四聚体(T)、二聚体(D)和单体(M)形式的浓度存在很大差异。与10名正常对照者的多克隆轻链相比,骨髓瘤患者中导致高D/M比值的D形式BJ蛋白比例显著更高。然而,多发性骨髓瘤患者的肾功能与D/M比值或BJ蛋白四聚体无关。这表明BJ蛋白的肾毒性与血清中BJ蛋白的聚合无关。在3例肾功能迅速下降的患者中发现了异常大小的BJ蛋白。其中2例患者的组织学检查显示有结节性肾小球硬化。

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