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单克隆角膜丙种球蛋白病:地形学考量

Monoclonal corneal gammopathy: topographic considerations.

作者信息

Sekundo W, Seifert P

机构信息

Department of Ophthalmology, University Eye Hospital, University of Bonn, Germany.

出版信息

Ger J Ophthalmol. 1996 Sep;5(5):262-7.

PMID:8911947
Abstract

Desposition of immunoglobulins in the cornea occasionally occurs in benign and malignant lymphoproliferative conditions. A 52-year-old woman with recently discovered monoclonal gammopathy of undetermined significance (MGUS) was referred to our hospital. Slit-lamp and ultrasound biomicroscopy revealed bilateral deposits within all corneal layers. The precipitates were organized in a circle, leaving a perilimbal zone and the axial cornea clear. Light microscopy of a biopsy disclosed confluent subepithelial deposits and defects in Bowman's layer. Immunoperoxidase reaction was positive only for IgG and IgG-kappa. Transmission electron microscopy confirmed the presence of extracellular rectangular and arcuate immunoglobulin crystalloids with a 10-nm periodicity but a non-crystalline defraction pattern. A review of the literature showed that the circumferential pattern of immunoglobulin deposition is associated with short-term visual symptoms and good visual acuity. The present report supports a hypothesis of immunoglobulin deposition via the limbal arcade and contradicts the "tear theory."

摘要

免疫球蛋白在角膜中的沉积偶尔会发生在良性和恶性淋巴增殖性疾病中。一名52岁近期被发现患有意义未明的单克隆丙种球蛋白病(MGUS)的女性被转诊至我院。裂隙灯和超声生物显微镜检查发现角膜各层均有双侧沉积物。沉淀物呈环状排列,周边角膜缘区和角膜中央区无沉积物。活检组织的光学显微镜检查显示上皮下沉积物融合且Bowman层有缺损。免疫过氧化物酶反应仅对IgG和IgG-κ呈阳性。透射电子显微镜证实存在细胞外矩形和弓形免疫球蛋白晶体,其具有10纳米的周期性,但衍射图案为非晶体状。文献回顾表明,免疫球蛋白沉积的圆周模式与短期视觉症状和良好视力相关。本报告支持通过角膜缘血管弓进行免疫球蛋白沉积的假说,并与“泪液理论”相矛盾。

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