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肾脏轻链沉积病:一例报告

Light-chain deposition disease of the kidney: a case report.

作者信息

Darouich Sihem, Goucha Rym, Jaafoura Mohamed Habib, Zekri Semy, Kheder Adel, Maiz Hedi Ben

机构信息

Electron Microscopy Laboratory, Faculty of Medicine of Tunis, Tunis, Tunisia.

出版信息

Ultrastruct Pathol. 2012 Apr;36(2):134-8. doi: 10.3109/01913123.2011.642464.

Abstract

A 41-year-old man was admitted for evaluation of nephrotic syndrome associated with microhematuria, hypertension, and moderate renal failure. In serum and urine samples, monoclonal IgG-lambda was detected. Bone marrow examination showed normal representation of all cell lines with normal range of plasma cells. Renal biopsy demonstrated diabetes-like nodular glomerulosclerosis. Immunofluorescence failed to demonstrate the presence of kappa or lambda light chains in the kidney. Electron microcopy showed granular electron-dense deposits along the glomerular basement membranes and in the mesangial nodules. The patient was diagnosed as having light-chain deposition disease (LCDD) without evidence of plasma cell dyscrasia. This report was designed to stress the significant challenges that remain in the diagnosis of LCDD-related glomerulopathy. The salient morphological features that help in making an accurate diagnosis are discussed.

摘要

一名41岁男性因评估与镜下血尿、高血压和中度肾衰竭相关的肾病综合征入院。在血清和尿液样本中检测到单克隆IgG-λ。骨髓检查显示所有细胞系表现正常,浆细胞范围正常。肾活检显示糖尿病样结节性肾小球硬化。免疫荧光未能在肾脏中显示κ或λ轻链的存在。电子显微镜检查显示沿肾小球基底膜和系膜结节有颗粒状电子致密沉积物。该患者被诊断为轻链沉积病(LCDD),无浆细胞异常增生的证据。本报告旨在强调在LCDD相关肾小球病诊断中仍然存在的重大挑战。讨论了有助于准确诊断的显著形态学特征。

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