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特发性本-周蛋白尿的转归

The outcome of idiopathic Bence Jones proteinuria.

作者信息

Kanoh T, Ohnaka T, Uchino H, Fujii H

出版信息

Tohoku J Exp Med. 1987 Jan;151(1):121-6. doi: 10.1620/tjem.151.121.

Abstract

The authors studied two patients with idiopathic Bence Jones proteinuria (BJP) that fulfill all the criteria proposed by Kyle and Greipp. None had evidence of overt multiple myeloma, of its variants, of primary systemic amyloidosis, or of other lymphoid tumors. In a patient with kappa type idiopathic BJP an elevation of a labelling index was found when an evolving myeloma developed 2 years later. The other had benign lambda type BJP until he died of bronchogenic carcinoma after 14 years. In most of patients with idiopathic BJP overt multiple myeloma or systemic amyloidosis have developed after a long period. An elevation of labelling index in the course of illness is expected to be a premonitory sign for malignant transformation. Idiopathic BJP may be characterized by less nephrotoxicity or amyloidogenicity of Bence Jones protein synthesized as well as a slow growth rate of tumor cells.

摘要

作者研究了两名符合凯尔(Kyle)和格雷普(Greipp)提出的所有标准的特发性本-周蛋白尿(BJP)患者。两人均无明显多发性骨髓瘤、其变体、原发性系统性淀粉样变性或其他淋巴瘤的证据。在一名κ型特发性BJP患者中,两年后发生进展性骨髓瘤时发现标记指数升高。另一名患者患有良性λ型BJP,14年后死于支气管源性癌。在大多数特发性BJP患者中,长期后会发生明显的多发性骨髓瘤或系统性淀粉样变性。病程中标记指数升高预计是恶性转化的先兆。特发性BJP的特征可能是所合成的本-周蛋白肾毒性或淀粉样变性较小,以及肿瘤细胞生长缓慢。

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