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克隆性浆细胞的直接浸润导致意义未明的单克隆丙种球蛋白病患者出现肾功能不全。

Direct infiltration of clonal plasma cells causes renal insufficiency in patients with monoclonal gammopathy of undetermined significance.

作者信息

Naka Ryosuke, Shimomura Yoshimitsu, Kanda-Kato Madoka, Hiramoto Nobuhiro, Hara Shigeo, Ishikawa Takayuki

机构信息

Department of Hematology Kobe City Hospital Organization Kobe City Medical Center General Hospital Kobe Japan.

Department of Social and Environmental Medicine Graduate School of Medicine Osaka University Osaka Japan.

出版信息

EJHaem. 2023 Jun 26;4(3):829-832. doi: 10.1002/jha2.747. eCollection 2023 Aug.

Abstract

Monoclonal gammopathy of undetermined significance (MGUS) is a benign but precancerous condition that can progress to multiple myeloma. Patients with MGUS are typically monitored closely for signs of disease progression, but in some cases, they may also develop renal insufficiency, a condition known as monoclonal gammopathy of renal significance (MGRS). In MGRS, M-protein secreted by a nonmalignant or premalignant cell clone triggers renal damage by definition. Herein, we report a case of a 66-year-old Asian male with MGUS complicated by renal insufficiency. A kidney biopsy showed no evidence of renal injury mediated by M-protein; instead, the direct infiltration of clonal cells into renal tissues was observed. Although five similar cases have been previously reported, our case is unique in that the involvement of clonal cells was directly confirmed by fluorescence in situ hybridization. Our findings suggest the need to consider a novel disease concept, as this phenomenon appears to be reproduced.

摘要

意义未明的单克隆丙种球蛋白病(MGUS)是一种良性但癌前状态,可进展为多发性骨髓瘤。MGUS患者通常会被密切监测疾病进展迹象,但在某些情况下,他们也可能发展为肾功能不全,即所谓的具有肾意义的单克隆丙种球蛋白病(MGRS)。在MGRS中,根据定义,由非恶性或癌前细胞克隆分泌的M蛋白会引发肾损伤。在此,我们报告一例66岁亚洲男性MGUS合并肾功能不全的病例。肾活检未显示M蛋白介导的肾损伤证据;相反,观察到克隆细胞直接浸润肾组织。尽管此前已有五例类似病例报道,但我们的病例独特之处在于通过荧光原位杂交直接证实了克隆细胞的浸润。我们的发现表明有必要考虑一种新的疾病概念,因为这种现象似乎会再次出现。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc89/10435693/720e5c69f867/JHA2-4-829-g001.jpg

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