与多发性骨髓瘤相关的结节性肾小球硬化。轻链等电点的作用。

Nodular glomerulosclerosis associated with multiple myeloma. Role of light chain isoelectric point.

作者信息

Palant C E, Bonitati J, Bartholomew W R, Brentjens J R, Walshe J J, Bentzel C J

出版信息

Am J Med. 1986 Jan;80(1):98-102. doi: 10.1016/0002-9343(86)90055-0.

Abstract

A 68-year-old female patient with multiple myeloma exhibited advanced nodular glomerulosclerosis. Immunofluorescence of the kidney showed kappa light chain deposition in the mesangium and in glomerular and tubular basement membrane. Isoelectric focusing and immunofixation of urinary proteins revealed an isolated kappa light chain with an unusually high isoelectric point of 8.4. Most light chain proteins have isoelectric points in the 4.6 to 6.7 range. Since loss of fixed negative charges may precede experimental glomerulosclerosis, it is proposed that this cationic circulating kappa chain may have interacted with glomerular polyanion, thereby inducing a nodular sclerotic reaction leading to irreversible renal damage.

摘要

一名68岁的多发性骨髓瘤女性患者出现了晚期结节性肾小球硬化。肾脏免疫荧光显示κ轻链沉积于系膜以及肾小球和肾小管基底膜。尿蛋白的等电聚焦和免疫固定显示出一条孤立的κ轻链，其等电点异常高，为8.4。大多数轻链蛋白的等电点在4.6至6.7范围内。由于固定负电荷的丧失可能先于实验性肾小球硬化出现，因此有人提出这种阳离子循环κ链可能与肾小球多阴离子相互作用，从而引发结节性硬化反应，导致不可逆的肾损伤。