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异基因干细胞移植后与移植物抗宿主病相关的膜性肾小球病。2例报告并文献复习。

Membranous glomerulopathy associated with graft-versus-host disease following allogeneic stem cell transplantation. Report of 2 cases and review of the literature.

作者信息

Lin J, Markowitz G S, Nicolaides M, Hesdorffer C S, Appel G B, D'Agati V D, Savage D G

机构信息

Division of Nephrology, Department of Medicine, Columbia University, New York-Presbyterian Hospital, New York, N.Y., USA.

出版信息

Am J Nephrol. 2001 Sep-Oct;21(5):351-6. doi: 10.1159/000046273.

Abstract

We report two cases of nephrotic syndrome presenting 18 and 20 months after allogeneic stem cell transplantation (alloSCT) with chronic myelogenous leukemia. Both patients had acute and chronic graft-versus-host disease (GVHD) and renal biopsy findings of membranous glomerulopathy (MG). A review of the literature revealed 10 additional cases of immune-complex-mediated glomerular disease following alloSCT, 8 of which were diagnostic of MG. All patients showed evidence of acute or chronic GVHD. Patients typically presented with preserved renal function (mean creatinine 1.2 mg/dl) and full nephrotic syndrome including heavy proteinuria (mean 9.2 g/24 h), edema, hypoalbuminemia (mean 2.1 g/dl) and hypercholesterolemia (mean 472 mg/dl). Most patients showed stabilization of renal function and significant decreases in proteinuria when treated with steroids and/or cyclosporine. The close temporal association as well as evidence from murine models of GVHD support a pathogenetic association between GVHD and the development of MG.

摘要

我们报告了两例异基因干细胞移植(alloSCT)治疗慢性粒细胞白血病后18个月和20个月出现肾病综合征的病例。两名患者均患有急性和慢性移植物抗宿主病(GVHD),肾活检结果为膜性肾小球病(MG)。文献回顾显示,alloSCT后还有10例免疫复合物介导的肾小球疾病病例,其中8例诊断为MG。所有患者均有急性或慢性GVHD的证据。患者通常表现为肾功能保留(平均肌酐1.2mg/dl),并出现完全性肾病综合征,包括大量蛋白尿(平均9.2g/24小时)、水肿、低白蛋白血症(平均2.1g/dl)和高胆固醇血症(平均472mg/dl)。大多数患者在接受类固醇和/或环孢素治疗后肾功能稳定,蛋白尿显著减少。GVHD与MG发生之间在时间上的密切关联以及来自GVHD小鼠模型的证据支持了两者之间的发病机制关联。

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