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大剂量美法仑联合自体外周血干细胞移植3年后系膜轻链沉积物消退

Resolution of mesangial light chain deposits 3 years after high-dose melphalan with autologous peripheral blood stem cell transplantation.

作者信息

Harada K, Akai Y, Sakan H, Yamaguchi Y, Nakatani K, Iwano M, Saito Y

机构信息

First Department of Internal Medicine, Nara Medical University, Nara, Japan.

出版信息

Clin Nephrol. 2010 Nov;74(5):384-8.

Abstract

A 52-year-old woman was admitted to our hospital for treatment of nephrotic syndrome. Funduscopic findings showed fundal hemorrhage and soft exudates, and serologic analysis showed a monoclonal serum component that was identified as Bence Jones protein-k type. A bone marrow biopsy showed diffuse proliferation of atypical plasma cells, while a renal biopsy showed diffuse and nodular mesangial proliferation. Immunohistochemical staining confirmed the presence of k chains along the glomerular basement membrane and in mesangial areas. The patient was diagnosed as multiple myeloma (Bence Jones k type) with light chain deposition disease (LCDD). After high-dose melphalan and autologous peripheral blood stem cell transplantation (PBSCT), the multiple myeloma and nephrotic syndrome were in complete remission; her renal function was improved, but a renal biopsy performed 6 months after PBSCT showed the persistence of diffuse and nodular lesions. By contrast, a renal biopsy performed 3 years later showed complete resolution of the diffuse and nodular mesangial proliferation.

摘要

一名52岁女性因肾病综合征入院治疗。眼底检查发现眼底出血和软性渗出物,血清学分析显示单克隆血清成分,鉴定为本-周蛋白k型。骨髓活检显示非典型浆细胞弥漫性增生,而肾活检显示弥漫性和结节性系膜增生。免疫组化染色证实沿肾小球基底膜和系膜区存在k链。该患者被诊断为多发性骨髓瘤(本-周蛋白k型)合并轻链沉积病(LCDD)。经过大剂量美法仑和自体外周血干细胞移植(PBSCT)后,多发性骨髓瘤和肾病综合征完全缓解;她的肾功能得到改善,但PBSCT后6个月进行的肾活检显示弥漫性和结节性病变持续存在。相比之下,3年后进行的肾活检显示弥漫性和结节性系膜增生完全消退。

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