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κ轻链肾病。一项病理学研究。

Kappa light chain nephropathy. A pathologic study.

作者信息

Bangerter A R, Murphy W M

出版信息

Virchows Arch A Pathol Anat Histopathol. 1987;410(6):531-9. doi: 10.1007/BF00781689.

Abstract

Percutaneous renal biopsies from 4 patients with clinically unsuspected kappa light chain nephropathy were studied using light, immunofluorescence, and electron microscopy. The diagnosis in each case was established by demonstrating monoclonal kappa light chain deposits in basement membranes and basement membrane-like structures of glomeruli, tubules, and blood vessels by immunofluorescence microscopy. Characteristic electron dense deposits occurred in every case but the intensity and distribution of electron densities did not correlate with the immunofluorescence findings. When light chain aggregation occurred, as evidenced by the distribution of electron dense deposits, it was proportional to the amount of basement membrane-like material as if these immunoglobulins had a particular affinity for structures chemically related to basement membranes. Although active tubulointerstitial lesions were prominent in all biopsies, there was considerable variation in glomerular pathology with only 1 case exhibiting the typical nodular glomerulosclerosis. Correlation of the light, immunofluorescence, and electron microscopic findings in these cases suggests that the pathogenesis of kappa light chain nephropathy is related to light chain nephrotoxicity directed to basement membrane-like structures with subsequent alterations in hemodynamics and structural renal damage.

摘要

对4例临床未怀疑κ轻链肾病患者的经皮肾活检组织进行了光镜、免疫荧光和电镜研究。通过免疫荧光显微镜在肾小球、肾小管和血管的基底膜及基底膜样结构中证实单克隆κ轻链沉积,从而确诊每例病例。每例均出现特征性电子致密沉积物,但电子密度的强度和分布与免疫荧光结果不相关。当出现轻链聚集时,如电子致密沉积物的分布所示,其与基底膜样物质的量成正比,就好像这些免疫球蛋白对与基底膜化学相关的结构具有特殊亲和力。尽管所有活检中活动性肾小管间质病变均很突出,但肾小球病理变化很大,仅1例表现为典型的结节性肾小球硬化。这些病例的光镜、免疫荧光和电镜结果相关性提示,κ轻链肾病的发病机制与针对基底膜样结构的轻链肾毒性有关,随后伴有血流动力学改变和肾脏结构损伤。

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