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单克隆丙种球蛋白病患者的足细胞病:3例患者及文献综述

Podocytopathy in patients with monoclonal gammopathy: three patients and literature review.

作者信息

Ribas Andrés, Puche Adrián, Gimeno Javier, Sans Laia, Barrios Clara, Márquez Eva, Naranjo Dolores, Lloveras Belén, Lop Joan, Ramos Natàlia, Soler Maria José, Gabaldon Alejandra, Crespo Marta, Rodríguez Eva

机构信息

Nephrology Department, Hospital del Mar, Barcelona, Spain.

Pathology Department, Hospital del Mar, Barcelona, Spain.

出版信息

Clin Kidney J. 2021 Sep 28;15(3):417-424. doi: 10.1093/ckj/sfab176. eCollection 2022 Mar.

Abstract

BACKGROUND

Renal manifestations of monoclonal gammopathies are of increasing interest among nephrologists. Typical manifestations include light chain cast nephropathy, amyloidosis or renal damage mediated by monoclonal immunoglobulin deposition. Podocytopathies in the setting of an underlying monoclonal gammopathy constitute a rare manifestation of these diseases and, although being described in the literature, remain a challenge since most data derive from case reports.

METHODS

A retrospective review of the clinical data of Hospital del Mar and Hospital Vall d'Hebron was performed to identify patients with minimal change disease (MCD) or focal and segmental glomerulosclerosis (FSGS) in the setting of neoplasms that produce monoclonal (M) protein. Additionally, a literature review on this topic was performed. This study aims to describe the clinical characteristics and outcomes of these patients.

RESULTS

Three patients were identified to have podocytopathy and monoclonal gammopathy between the years 2013 and 2020. All three were males and  >65 years of age. Two patients were diagnosed with MCD and one patient was diagnosed with FSGS. All patients underwent a kidney biopsy and light and electron microscopic studies were performed. The underlying causes of monoclonal gammopathy were multiple myeloma in two cases and Waldeström macroglobulinemia in one case. Two patients developed nephrotic syndrome during the follow-up. All patients were under active hematological treatment. One patient presented a complete remission of proteinuria whereas the other two presented a partial remission.

CONCLUSIONS

Podocytopathies may infrequently be found in patients with monoclonal gammopathies. Patients with overt glomerular proteinuria and hematological disorders with M protein should undergo a kidney biopsy for prompt diagnosis and to specify a prognosis. In addition, further study on this matter must be done to understand the pathophysiology and treat these patients appropriately.

摘要

背景

单克隆丙种球蛋白病的肾脏表现日益受到肾脏病学家的关注。典型表现包括轻链管型肾病、淀粉样变性或由单克隆免疫球蛋白沉积介导的肾损伤。潜在单克隆丙种球蛋白病背景下的足细胞病是这些疾病的一种罕见表现,尽管文献中有描述,但由于大多数数据来自病例报告,仍然是一个挑战。

方法

对德尔马医院和瓦尔德希伯伦医院的临床资料进行回顾性分析,以确定在产生单克隆(M)蛋白的肿瘤背景下患有微小病变病(MCD)或局灶节段性肾小球硬化(FSGS)的患者。此外,对该主题进行了文献综述。本研究旨在描述这些患者的临床特征和结局。

结果

2013年至2020年间,3例患者被确诊为足细胞病和单克隆丙种球蛋白病。3例均为男性,年龄均>65岁。2例患者被诊断为MCD,1例患者被诊断为FSGS。所有患者均接受了肾活检,并进行了光镜和电镜检查。单克隆丙种球蛋白病的潜在病因在2例中为多发性骨髓瘤,1例中为华氏巨球蛋白血症。2例患者在随访期间出现肾病综合征。所有患者均接受积极的血液学治疗。1例患者蛋白尿完全缓解,另外2例患者部分缓解。

结论

单克隆丙种球蛋白病患者中可能很少发现足细胞病。有明显肾小球蛋白尿和伴有M蛋白的血液系统疾病的患者应进行肾活检以迅速诊断并明确预后。此外,必须对此问题进行进一步研究,以了解其病理生理学并对这些患者进行适当治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/666e/8862048/0f513e08b575/sfab176fig1.jpg

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