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一例慢性粒单核细胞相关溶菌酶诱导的肾病。

A case of chronic myelomonocytic related lysozyme-induced nephropathy.

作者信息

Kansal Abhik, Peiris Thanushi, Ramnarain Jaineel, Wang Joan, Daneshvar Simin, Chow Fiona, Fleming Shaun

机构信息

School of Medicine, Deakin University, 1 Gheringhap Street, Geelong, Victoria, 3220, Australia.

The Royal Melbourne Hospital, Melbourne, Australia.

出版信息

BMC Nephrol. 2025 Jul 1;26(1):302. doi: 10.1186/s12882-024-03895-2.

Abstract

BACKGROUND

Lysozyme-induced nephropathy (LyN) is a rare cause of acute kidney injury (AKI) in patients with chronic myelomonocytic leukemia (CMML). Diagnosing LyN is challenging due to the numerous potential and concurrent aetiologies. Previous case reports have demonstrated the diagnostic utility of serum and urinary lysozyme measurements, which may correlate with renal dysfunction; however, the role of renal imaging remains unclear and renal biopsy remains essential for diagnosis.

CASE PRESENTATION

An independent 80-year-old male presented to an Australian tertiary hospital with stage 3 AKI on a background of CMML previously treated with thioguanine and hydroxycarbamide. Renal tract ultrasound revealed expansile, non-vascular material bilaterally in renal collecting systems which was confirmed by renal biopsy to be lysozyme-induced nephropathy causing renal dysfunction. Despite escalated anti-leukaemic treatment he progressed to stage 4 chronic kidney disease contributing to increasing frailty and eventually pursued voluntary assisted dying after a fall with terminal traumatic injuries.

CONCLUSIONS

LyN is a rare cause of acute kidney injury amongst patients with chronic myelomonocytic leukaemia. This case highlights a rare instance of LyN and illustrates potential radiological markers that may aid in diagnosis. It also emphasizes the complexity of managing LyN in the context of underlying haematological malignancies.

摘要

背景

溶菌酶诱导性肾病(LyN)是慢性粒单核细胞白血病(CMML)患者急性肾损伤(AKI)的罕见病因。由于存在众多潜在病因和并发病因,LyN的诊断具有挑战性。既往病例报告显示血清和尿溶菌酶检测具有诊断价值,其可能与肾功能不全相关;然而,肾脏影像学检查的作用仍不明确,肾活检仍是诊断的关键。

病例介绍

一名80岁男性因CMML前来澳大利亚一家三级医院就诊,此前曾接受硫鸟嘌呤和羟基脲治疗,目前处于3期AKI。肾脏超声显示双侧肾集合系统有扩张性、无血管的物质,肾活检证实为溶菌酶诱导性肾病导致肾功能不全。尽管加强了抗白血病治疗,他仍进展为4期慢性肾脏病,导致身体日益虚弱,最终在一次跌倒并遭受致命创伤后选择了自愿协助死亡。

结论

LyN是慢性粒单核细胞白血病患者急性肾损伤的罕见病因。本病例突出了LyN的罕见情况,并说明了可能有助于诊断的潜在影像学标志物。它还强调了在存在潜在血液系统恶性肿瘤的情况下管理LyN的复杂性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4a17/12210450/0fb9f07b5cba/12882_2024_3895_Fig1_HTML.jpg

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