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系统性κ轻链沉积。一项超微结构和免疫组织化学研究。

Systemic kappa light-chain deposition. An ultrastructural and immunohistochemical study.

作者信息

Linder J, Croker B P, Vollmer R T, Shelburne J

出版信息

Am J Surg Pathol. 1983 Jan;7(1):85-93. doi: 10.1097/00000478-198301000-00009.

Abstract

This report describes the pathology of kappa light-chain deposition in a 55-year-old patient who presented with respiratory insufficiency and hepatomegaly. Biopsies of lung and liver showed PAS-positive deposits which did not stain with congo red, crystal violet, or thioflavin-T. By indirect immunoperoxidase techniques, the deposits were composed of kappa light-chain immunoglobin. Electron microscopy revealed granular and fibrillar electron-dense material which lacked the characteristics of amyloid. Subsequent clinical studies showed this patient had a plasma cell dyscrasia. These data show that kappa light-chain deposition is not limited to the kidney, and that the first manifestation of a plasma cell dyscrasia may be systemic deposits of light chain. These deposits can be distinguished from amyloid by their immunochemical, tinctorial, and ultrastructural appearance.

摘要

本报告描述了一名55岁患者的κ轻链沉积病理情况,该患者表现为呼吸功能不全和肝肿大。肺和肝活检显示PAS阳性沉积物,刚果红、结晶紫或硫黄素-T染色均为阴性。通过间接免疫过氧化物酶技术,沉积物由κ轻链免疫球蛋白组成。电子显微镜显示有颗粒状和纤维状电子致密物质,缺乏淀粉样蛋白的特征。随后的临床研究表明该患者患有浆细胞异常增殖症。这些数据表明κ轻链沉积并不局限于肾脏,浆细胞异常增殖症的首发表现可能是轻链的系统性沉积。这些沉积物可通过其免疫化学、染色和超微结构外观与淀粉样蛋白相区分。

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