轻链沉积病患者肾脏组织学的五次连续评估

Five Sequential Evaluations of Renal Histology in a Patient with Light Chain Deposition Disease.

作者信息

Ueno Toshiharu, Kikuchi Koichi, Hazue Ryo, Mise Koki, Sumida Keiichi, Hayami Noriko, Suwabe Tatsuya, Hoshino Junichi, Sawa Naoki, Arizono Kenji, Hara Shigeko, Takaichi Kenmei, Fujii Takeshi, Ohashi Kenichi, Ubara Yoshifumi

机构信息

Nephrology Center, Toranomon Hospital, Japan.

出版信息

Intern Med. 2016;55(20):2993-2999. doi: 10.2169/internalmedicine.55.6213. Epub 2016 Oct 15.

DOI:10.2169/internalmedicine.55.6213

PMID:27746438

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5109568/

Abstract

A 58-year-old man was referred to our institution for an evaluation of nephrotic range proteinuria. Renal biopsy showed a marked expansion of the mesangial matrix and thickening of glomerular basement membrane (GBM) in periodic acid-silver methenamine (PAM). Immunofluorescence (IF) revealed strong staining for the monoclonal kappa light chain. EM demonstrated massive subendothelial and mesangial dense deposits. As a result, light chain deposition disease (LCDD) was diagnosed. Melphalan and prednisolone (MP) therapy was started, which was continued for 10 years with minimal complications. Serial evaluations of renal histology revealed the resolution of nodular lesions and the glomeruli became nearly normal. MP therapy can therefore be an effective therapeutic option for LCDD if it is continued over the long term.

摘要

一名58岁男性因肾病范围蛋白尿评估被转诊至我院。肾活检显示在高碘酸-银甲胺（PAM）染色中系膜基质显著扩张和肾小球基底膜（GBM）增厚。免疫荧光（IF）显示单克隆κ轻链强染色。电镜（EM）显示大量内皮下和系膜致密沉积物。因此，诊断为轻链沉积病（LCDD）。开始使用美法仑和泼尼松龙（MP）治疗，持续10年，并发症极少。肾脏组织学的系列评估显示结节性病变消退，肾小球几乎恢复正常。因此，如果长期持续使用，MP治疗可能是LCDD的一种有效治疗选择。