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一例伴有肾脏受累的POEMS综合征的M蛋白阴性患者的病例报告。

A case report of an M protein-negative patient with POEMS syndrome associated with renal involvement.

作者信息

Liu Wenlin, Zhou Yue, Li Lingyan, Zhang Fan, Xiong Zuying, Hou Shuang

机构信息

Department of Nephrology, Peking University Shenzhen Hospital, Shenzhen, Guangdong Province, 518000, China.

出版信息

BMC Nephrol. 2024 Dec 24;25(1):472. doi: 10.1186/s12882-024-03898-z.

DOI:10.1186/s12882-024-03898-z
PMID:39719549
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11668054/
Abstract

BACKGROUND

POEMS syndrome with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes is an uncommon plasma cell paraneoplastic syndrome involving multiple system. It is relatively rare in clinical practice, and renal involvement is a usual yet easily overlooked symptom.

CASE PRESENTATION

We successfully treated a patient with M protein-negative POEMS syndrome with membranoproliferative glomerulonephritis (MPGN) findings and thrombotic microangiopathic changes by comparing the level of Vascular endothelial growth factor (VEGF) in the serum and the changes in polyserositis before and after the patient's treatment.

CONCLUSION

POEMS syndrome clinically involves multiple systems and has complex symptoms. Because of the diversity of the disease manifestations, identification of atypical POEMS syndrome and timely intervention are important for patient survival and prognosis.

摘要

背景

POEMS综合征伴有多发性神经病、器官肿大、内分泌病、M蛋白和皮肤改变,是一种累及多系统的罕见浆细胞副肿瘤综合征。在临床实践中相对少见,肾脏受累是常见但易被忽视的症状。

病例报告

通过比较患者治疗前后血清中血管内皮生长因子(VEGF)水平及多浆膜炎的变化,我们成功治疗了一名患有膜增生性肾小球肾炎(MPGN)表现和血栓性微血管病变的M蛋白阴性POEMS综合征患者。

结论

POEMS综合征临床累及多个系统且症状复杂。由于疾病表现的多样性,识别非典型POEMS综合征并及时干预对患者的生存和预后很重要。

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本文引用的文献

1
Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome with dilated cardiomyopathy: A case report.多神经病、器官肿大、内分泌病、M蛋白、皮肤改变综合征伴扩张型心肌病:一例报告。
World J Clin Cases. 2024 Jan 26;12(3):601-606. doi: 10.12998/wjcc.v12.i3.601.
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Update on the POEMS syndrome.POEMS综合征的最新进展。
Blood Res. 2022 Apr 30;57(S1):27-31. doi: 10.5045/br.2022.2022001.
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POEMS Syndrome with Biclonal Gammopathy and Renal Involvement.POEMS 综合征伴双克隆丙种球蛋白血症和肾脏受累。
Intern Med. 2022 Jul 15;61(14):2191-2196. doi: 10.2169/internalmedicine.8714-21. Epub 2022 Mar 12.
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Bortezomib plus dexamethasone as first-line therapy for patients with POEMS syndrome.硼替佐米联合地塞米松作为 POEMS 综合征患者的一线治疗。
Ann Hematol. 2021 Nov;100(11):2755-2761. doi: 10.1007/s00277-021-04609-6. Epub 2021 Jul 31.
5
Update on the Diagnosis and Treatment of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome: A Review.POEMS(多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变)综合征的诊断和治疗进展:综述。
JAMA Oncol. 2021 Sep 1;7(9):1383-1391. doi: 10.1001/jamaoncol.2021.0586.
6
Exploring the Intracrine Functions of VEGF-A.探索血管内皮生长因子-A的自分泌功能。
Biomolecules. 2021 Jan 19;11(1):128. doi: 10.3390/biom11010128.
7
High rates of venous and arterial thrombotic events in patients with POEMS syndrome: results from the UCLH (UK) POEMS Registry.POEMS综合征患者静脉和动脉血栓形成事件的高发生率:来自英国伦敦大学学院医院(UCLH)POEMS登记处的结果
Blood Adv. 2020 May 26;4(10):2139-2142. doi: 10.1182/bloodadvances.2020001492.
8
POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management.POEMS 综合征:2019 年关于诊断、风险分层和管理的更新。
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9
VEGF in Signaling and Disease: Beyond Discovery and Development.血管内皮生长因子在信号转导和疾病中的作用:超越发现和开发。
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