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[具有肾脏意义的单克隆丙种球蛋白病与膜增生性肾小球肾炎:一种关系复杂但具有潜在治疗机会的疾病]

[Monoclonal gammopathy of renal significance and membranoproliferative glomerulonephritis: a complex relationship with promising therapeutic opportunities].

作者信息

Manes Massimo, Radin Elisabetta, Pellu' Valentina, Molino A Andrea, Gabrielli Danila, Parodi Emanuele, Caputo Donatella, Paternoster Giuseppe, Barreca Antonella, Pagliaro Maria, Quattrocchio Giacomo, Roccatello Dario

机构信息

S.C. Nefrologia e Dialisi. Ospedale "Umberto "Parini", Viale Ginevra 1. Aosta.

S.C.D.U. Anatomia e Istologia Patologica 1 U. AOU Città della Salute e della Scienza. Ospedale Molinette. Torino.

出版信息

G Ital Nefrol. 2019 Jul 24;36(4):2019-vol4.

Abstract

In the last few years, the increasing awareness of the complex interaction between monoclonal component and renal damage has determined not only a new classification of the associated disorders, called Monoclonal Gammopathy of Renal Significance (MGRS), but has also contributed to emphasize the importance of an early diagnosis of the renal involvement, which is often hard to detect but can evolve towards terminal uraemia; it has also pointed at the need to treat these disorders with aggressive regimens, even if they are not strictly neoplastic. The case described here presented urinary abnormalities and renal failure secondary to a membranoproliferative glomerulonephritis (MPGN), with intensively positive immunofluorescence (IF) for monoclonal k light chain and C3, and in the absence of a neoplastic lympho-proliferative disorder documented on bone marrow biopsy. After the final diagnosis of MGRS, the patient was treated with several cycles of a therapy including dexamethasone, cyclophosphamide and bortezomib, showing a good functional and clinical response.

摘要

在过去几年中,人们对单克隆成分与肾损伤之间复杂相互作用的认识不断提高,这不仅促成了相关疾病的新分类,即具有肾意义的单克隆丙种球蛋白病(MGRS),还促使人们强调早期诊断肾脏受累的重要性,肾脏受累往往难以察觉,但可能发展为终末期尿毒症;这也表明即使这些疾病并非严格意义上的肿瘤性疾病,也需要采用积极的治疗方案进行治疗。此处描述的病例表现为继发于膜增生性肾小球肾炎(MPGN)的尿液异常和肾衰竭,免疫荧光(IF)显示单克隆κ轻链和C3呈强阳性,且骨髓活检未发现肿瘤性淋巴增殖性疾病。在最终诊断为MGRS后,该患者接受了包括地塞米松、环磷酰胺和硼替佐米在内的多个疗程治疗,显示出良好的功能和临床反应。

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