类固醇抵抗性肾病综合征作为Waldenström巨球蛋白血症的副肿瘤综合征1例。

IF 1 Q4 UROLOGY & NEPHROLOGY
Konosuke Hirano, Sayuri Shirai, Teppei Koyama, Ryuichiro Makinouchi, Shinji Machida, Katsuomi Matsui, Shigeki Kosugi, Yasushi Ariizumi, Yukiko Kanetsuna, Junki Koike, Naohiko Imai
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引用次数: 0

摘要

与淋巴增生性疾病相关的肾小球肾炎的报道很常见,但伴随非霍奇金淋巴瘤的微小改变病(MCD)的报道很少见。在这里,我们提出了一个45岁的女性在MCD治疗期间被诊断为原发性Waldenström巨球蛋白血症(WM)的病例。肾活检显示MCD部分内皮细胞损伤。随后,她出现类固醇抵抗性肾病综合征和暂时性急性肾损伤,需要透析。在苯达莫司汀和利妥昔单抗联合治疗WM后,肾病综合征得到缓解。肾脏组织学检查结果和治疗过程提示MCD和WM之间的因果关系。MCD与WM相关的发病机制可能与B淋巴细胞衍生的肾小球通透性因子的释放有关。虽然轻度WM通常通过观察治疗,但与WM相关的类固醇抵抗性肾病综合征应引起对副肿瘤综合征的怀疑,需要针对WM的积极化疗作为关键的治疗方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Steroid-resistant nephrotic syndrome as paraneoplastic syndrome of Waldenström macroglobulinemia: a case report.

Reports of glomerulonephritis associated with lymphoproliferative disorders are common, but reports of minimal change disease (MCD) accompanying non-Hodgkin's lymphoma are rare. Here, we present a case of a 45-year-old woman diagnosed with primary Waldenström's macroglobulinemia (WM) during MCD treatment. Her kidney biopsy revealed endothelial cell injury in parts of the MCD. Subsequently, she developed steroid-resistant nephrotic syndrome and temporary acute kidney injury, requiring dialysis. Remission of the nephrotic syndrome was achieved after initiating combination therapy with bendamustine and rituximab for WM. The renal histological findings and treatment course suggest a causal relationship between MCD and WM in this case. The pathogenesis of MCD associated with WM may involve the release of glomerular permeability factors derived from B lymphocytes. Although mild WM is often managed with observation, steroid-resistant nephrotic syndrome associated with WM should raise suspicion of a paraneoplastic syndrome, necessitating active chemotherapy targeting WM as a critical treatment approach.

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来源期刊
CEN Case Reports
CEN Case Reports UROLOGY & NEPHROLOGY-
CiteScore
1.90
自引率
0.00%
发文量
80
期刊介绍: Clinical and Experimental Nephrology (CEN) Case Reports is a peer-reviewed online-only journal, officially published biannually by the Japanese Society of Nephrology (JSN).  The journal publishes original case reports in nephrology and related areas.  The purpose of CEN Case Reports is to provide clinicians and researchers with a forum in which to disseminate their personal experience to a wide readership and to review interesting cases encountered by colleagues all over the world, from whom contributions are welcomed.
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