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以皮疹为首发症状的继发性肾病综合征的诊断与治疗:病例报告。

Diagnosis and treatment of secondary nephrotic syndrome with rash as the first symptom: a case report.

机构信息

Department of Nephrology, Tianyou Hospital, Wuhan University of Science and Technology, Wuhan, China.

Department of Nephrology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1095 Jiefang Ave, Wuhan, 430030, China.

出版信息

BMC Nephrol. 2024 Jul 15;25(1):225. doi: 10.1186/s12882-024-03665-0.

Abstract

BACKGROUND

Membranous nephropathy (MN) is a common type of nephrotic syndrome (NS) in adults, accounting for about 20-30% of cases. Although secondary to specific factors, the coexistence of MN and mantle cell lymphoma (MCL) has been scarcely reported in clinical literature.

CASE PRESENTATION

A 59-year-old Chinese male was admitted to the hospital with a generalized pruritic rash with bilateral lower extremity edema, which did not improve significantly after symptomatic treatment. He had undergone renal biopsy, and the diagnosis was thought to be secondary MN (SMN), therefore, we did a lymph node biopsy on the patient and found that MN was complicated with MCL. Soon after, the patient was admitted to the hematology department for a BR chemotherapy regimen (composed of bendamustine 90 mg/m BSA (body surface area), rituximab 375 mg/m BSA and dexamethasone 5 mg), and during the post-treatment follow-up, both his symptoms and renal function improved.

CONCLUSIONS

The mechanism underlying the combination of SMN and MCL remains elusive and exceedingly rare, consequently often overlooked in clinical practice. This case serves to offer valuable clinical insights for diagnosis and treatment, while emphasizing the pivotal role of renal pathology in clinical assessment.

摘要

背景

膜性肾病(MN)是成人肾病综合征(NS)的常见类型,约占 20-30%。尽管继发于特定因素,但在临床文献中,MN 与套细胞淋巴瘤(MCL)共存的情况很少见。

病例介绍

一名 59 岁的中国男性因全身性瘙痒性皮疹伴双侧下肢水肿入院,经对症治疗后症状改善不明显。他曾接受过肾脏活检,诊断为继发性 MN(SMN),因此我们对患者进行了淋巴结活检,发现 MN 并发 MCL。随后,患者被收入血液科接受 BR 化疗方案(由苯达莫司汀 90mg/m2(体表面积)、利妥昔单抗 375mg/m2和地塞米松 5mg 组成),在治疗后随访期间,患者的症状和肾功能均有所改善。

结论

SMN 与 MCL 合并的机制尚不清楚,且极为罕见,因此在临床实践中经常被忽视。本病例为诊断和治疗提供了有价值的临床见解,并强调了肾脏病理学在临床评估中的关键作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/068d/11247727/f3861fca8efe/12882_2024_3665_Fig1_HTML.jpg

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