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良性单克隆丙种球蛋白病中的角膜后晶体沉积物:一例临床病理病例报告

Posterior corneal crystalline deposits in benign monoclonal gammopathy: a clinicopathologic case report.

作者信息

Rodrigues M M, Krachmer J H, Miller S D, Newsome D A

出版信息

Arch Ophthalmol. 1979 Jan;97(1):124-8. doi: 10.1001/archopht.1979.01020010058014.

Abstract

A 74-year-old woman had bilateral, deep stromal, patchy crystalline corneal deposits with the greatest density in the midperiphery. Visual acuity was 6/120 in the right eye and finger counting at 1 m in the left eye. Histological examination of the corneal button showed large, irregular amorphous masses in the posterior stroma. The deposits stained red with Masson's trichrome and were positive for protein with the Danielli stain. Stains for amyloid, copper, and lipid were negative. The immunoperoxidase stain was positive for polyvalent IgG and kappa light chains. Transmission electron microscopy disclosed electron-dense deposits with linear and honeycomb profiles. Laboratory investigations disclosed elevated serum and urinary IgG kappa light chain (Bence Jones protein) levels. Urinary amino acids were normal. The serum copper level was elevated. Antinuclear antibody was positive at a titer of 1:80. A bone marrow aspirate was normal, as were roentgenograms of the skull.

摘要

一名74岁女性双眼角膜基质深层有散在的结晶样沉积物,中周边部密度最高。右眼视力为6/120,左眼在1米处仅能数指。角膜植片的组织学检查显示后基质中有大的、不规则的无定形团块。这些沉积物经Masson三色染色呈红色,经Danielli染色蛋白质呈阳性。淀粉样蛋白、铜和脂质染色均为阴性。免疫过氧化物酶染色显示多价IgG和κ轻链呈阳性。透射电子显微镜显示有电子致密沉积物,呈线性和蜂窝状形态。实验室检查发现血清和尿中IgG κ轻链(本周氏蛋白)水平升高。尿氨基酸正常。血清铜水平升高。抗核抗体滴度为1:80时呈阳性。骨髓穿刺正常,颅骨X线片也正常。

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