Hirano Konosuke, Shirai Sayuri, Koyama Teppei, Makinouchi Ryuichiro, Machida Shinji, Matsui Katsuomi, Kosugi Shigeki, Ariizumi Yasushi, Kanetsuna Yukiko, Koike Junki, Imai Naohiko
Division of Nephrology and Hypertension, Department of Internal Medicine, St. Marianna University School of Medicine, Yokohama City Seibu Hospital, Yokohama, Japan.
Department of Nephrology, Shin-Yurigaoka General Hospital, Kawasaki, Japan.
CEN Case Rep. 2025 Jan 14. doi: 10.1007/s13730-025-00968-5.
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.
肾小球肾炎与淋巴增殖性疾病相关的报道很常见，但非霍奇金淋巴瘤伴发微小病变病（MCD）的报道却很罕见。在此，我们报告一例45岁女性病例，该患者在MCD治疗期间被诊断为原发性华氏巨球蛋白血症（WM）。她的肾活检显示MCD部分存在内皮细胞损伤。随后，她发展为类固醇抵抗型肾病综合征和暂时性急性肾损伤，需要进行透析。在开始使用苯达莫司汀和利妥昔单抗联合治疗WM后，肾病综合征得以缓解。肾脏组织学检查结果和治疗过程表明，该病例中MCD与WM之间存在因果关系。与WM相关的MCD的发病机制可能涉及源自B淋巴细胞的肾小球通透性因子的释放。尽管轻度WM通常通过观察进行管理，但与WM相关的类固醇抵抗型肾病综合征应引起对副肿瘤综合征的怀疑，有必要积极进行针对WM的化疗作为关键治疗方法。
