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免疫触须样肾小球病促成POEMS综合征的发现。

Immunotactoid glomerulopathy leading to the discovery of POEMS syndrome
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作者信息

Philipponnet Carole, Kemeny Jean-Louis, Garrouste Cyril, Soubrier Martin, Heng Anne-Elisabeth

出版信息

Clin Nephrol. 2017 Jun;87(6):310-315. doi: 10.5414/CN109056.

Abstract

Monoclonal gammopathy of renal significance (MGRS) can manifest in many different ways depending on the nature of the immunoglobulin and its physicochemical properties. MGRS can lead to the discovery of a hematological malignancy. We report the case of a 32-year-old female patient who underwent renal biopsy on account of an impure nephrotic syndrome associated with immunoglobulin (Ig)G κ monoclonal gammopathy. Histological analysis revealed membranoproliferative glomerulonephritis with IgG, IgM, κ, λ, and C3 deposits. Due to an unfavorable progression, a second renal biopsy was performed. Electron microscopy analysis revealed an immunotactoid glomerulopathy. At the same time, a POEMS syndrome diagnosis (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin abnormalities) was confirmed in light of the following: 1) IgG κ monoclonal gammopathy, 2) axonal neuropathy, 3) osteosclerosis, 4) melanoderma, 5) hepatosplenomegaly and adenopathies, 6) Castleman disease, and 7) edema. Our observation is the first case of immunotactoid glomerulopathy leading to the discovery of a POEMS syndrome. Renal involvement in POEMS syndrome typically exhibits a thrombotic microangiopathy-like membranoproliferative glomerulonephritis appearance associated with endothelial lesions stigmata. However, monoclonal immunoglobulin deposition disorder should be considered in the event of an atypical case. In this indication, electron microscopy is the examination of choice for assessing immunoglobulin deposition nephropathy.
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摘要

具有肾脏意义的单克隆丙种球蛋白病(MGRS)可根据免疫球蛋白的性质及其物理化学特性以多种不同方式表现出来。MGRS可导致血液系统恶性肿瘤的发现。我们报告了一例32岁女性患者,该患者因与免疫球蛋白(Ig)Gκ单克隆丙种球蛋白病相关的不纯肾病综合征接受了肾活检。组织学分析显示为膜增生性肾小球肾炎,伴有IgG、IgM、κ、λ和C3沉积。由于病情进展不利,进行了第二次肾活检。电子显微镜分析显示为免疫触须样肾小球病。同时,根据以下情况确诊为POEMS综合征(多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤异常):1)IgGκ单克隆丙种球蛋白病,2)轴索性神经病,3)骨硬化,4)黑皮病,5)肝脾肿大和淋巴结病,6)卡斯特曼病,7)水肿。我们的观察是首例因免疫触须样肾小球病而发现POEMS综合征的病例。POEMS综合征的肾脏受累通常表现为血栓性微血管病样膜增生性肾小球肾炎外观,伴有内皮病变特征。然而,在非典型病例中应考虑单克隆免疫球蛋白沉积病。在此情况下,电子显微镜检查是评估免疫球蛋白沉积性肾病的首选检查方法。

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