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单克隆免疫球蛋白沉积病:免疫球蛋白链改变的综述

Monoclonal immunoglobulin deposition disease: a review of immunoglobulin chain alterations.

作者信息

Preud'homme J L, Aucouturier P, Touchard G, Khamlichi A A, Rocca A, Denoroy L, Cogné M

机构信息

Laboratory of Immunology and Immunopathology (CNRS URA 1172, Immunology and Molecular Interactions), Poitiers, France.

出版信息

Int J Immunopharmacol. 1994 May-Jun;16(5-6):425-31. doi: 10.1016/0192-0561(94)90032-9.

Abstract

Monoclonal immunoglobulin deposition disease, a complication of overtly malignant or apparently benign immunoproliferative disorders, is a severe disease featuring tissue deposition of monoclonal light, light and heavy, or heavy chains. A number of converging arguments strongly suggest a direct pathogenetic role of structural abnormalities or peculiarities of variables regions of light and/or heavy chains (associated with deletions in the constant region for the heavy chains). Recent structural data on these abnormal immunoglobulin chains are reviewed.

摘要

单克隆免疫球蛋白沉积病是明显恶性或看似良性的免疫增殖性疾病的一种并发症,是一种严重疾病,其特征为单克隆轻链、轻链和重链或重链在组织中沉积。大量相互印证的论据有力地表明,轻链和/或重链可变区的结构异常或特性(与重链恒定区缺失相关)具有直接致病作用。本文综述了这些异常免疫球蛋白链的最新结构数据。

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