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最初诊断为血管外容量负荷增加的POEMS综合征的临床特征:一项单中心回顾性研究

[Clinical characteristics of POEMS syndrome initially diagnosed as increased extravascular volume load: a single-center retrospective study].

作者信息

Zhang T, Zhang J, Shen X X, Jin Y Y, Zhang R, Li J Y, Chen L J

机构信息

Department of Hematology, the First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing 210029, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2024 Jul 14;45(7):694-697. doi: 10.3760/cma.j.cn121090-20231117-00264.

DOI:10.3760/cma.j.cn121090-20231117-00264
PMID:39231776
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11388126/
Abstract

POEMS syndrome is a rare plasma cell dysplasia. Its clinical manifestations include polyneuropathy, monoclonal protein, increased extravascular volume load, endocrinopathy, organomegaly and skin changes. The complex and atypical symptoms at presentation make early diagnosis challenging due to multiple system involvement. Peripheral neuropathy, limb numbness, is the most common initial symptom of this disease. However, case reports of increased extravascular volume load are rare. This article collected and analyzed the clinical data of two groups of patients with different initial symptoms (increased extravascular volume load and limb numbness). The clinical characteristics and treatment responses were summarized.

摘要

POEMS综合征是一种罕见的浆细胞发育异常疾病。其临床表现包括多发性神经病、单克隆蛋白、血管外容量负荷增加、内分泌病、器官肿大和皮肤改变。由于多系统受累,该疾病呈现出的复杂且不典型症状使得早期诊断具有挑战性。周围神经病变、肢体麻木是该疾病最常见的初始症状。然而,血管外容量负荷增加的病例报告较为罕见。本文收集并分析了两组具有不同初始症状(血管外容量负荷增加和肢体麻木)患者的临床资料。总结了其临床特征及治疗反应。

相似文献

1
[Clinical characteristics of POEMS syndrome initially diagnosed as increased extravascular volume load: a single-center retrospective study].最初诊断为血管外容量负荷增加的POEMS综合征的临床特征:一项单中心回顾性研究
Zhonghua Xue Ye Xue Za Zhi. 2024 Jul 14;45(7):694-697. doi: 10.3760/cma.j.cn121090-20231117-00264.
2
Atypical polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes syndrome without polyneuropathy: A case report.非典型多发性神经病、器官肿大、内分泌病、单克隆蛋白及无多发性神经病的皮肤改变综合征:一例报告
Medicine (Baltimore). 2020 Jul 2;99(27):e20812. doi: 10.1097/MD.0000000000020812.
3
Castleman disease of the hyaline vascular variant transforming to POEMS syndrome as endpoint: a case report.以透明血管型Castleman病转变为POEMS综合征为终点:一例病例报告
BMC Neurol. 2018 Oct 9;18(1):169. doi: 10.1186/s12883-018-1172-7.
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Kennedy Disease Misdiagnosed as Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) Syndrome: A Case Report.肯尼迪病误诊为多神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变(POEMS)综合征:一例报告
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Living with Polyneuropathy Organomegaly Endocrinopathy Monoclonal gammopathy Skin changes (POEMS) syndrome: a case study of healthcare experiences and quality of life.患有多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白血症、皮肤改变(POEMS)综合征:一例医疗体验和生活质量的案例研究。
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Endocrine Manifestations in POEMS Syndrome: a case report and literature review.POEMS 综合征的内分泌表现:病例报告及文献复习。
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POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome in China.中国的 POEMS(多发性神经病、器官肿大、内分泌病、M 蛋白和皮肤改变)综合征。
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Clinicopathological profile of polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome.多发性神经病、器官肿大、内分泌病、M 蛋白和皮肤改变(POEMS)综合征的临床病理特征。
J Clin Neurosci. 2011 Mar;18(3):356-60. doi: 10.1016/j.jocn.2010.07.124. Epub 2011 Jan 21.

本文引用的文献

1
Efficacy and Safety of Regimens Used for the Treatment of POEMS Syndrome- A Systematic Review.
Clin Lymphoma Myeloma Leuk. 2022 Jan;22(1):e26-e33. doi: 10.1016/j.clml.2021.07.033. Epub 2021 Aug 2.
2
Treatment and outcomes of POEMS syndrome: changes in the past 20 years.POEMS综合征的治疗与预后:过去20年的变化
Blood Cancer J. 2021 Aug 14;11(8):145. doi: 10.1038/s41408-021-00540-1.
3
POEMS syndrome: 2021 Update on diagnosis, risk-stratification, and management.POEMS 综合征:诊断、风险分层和治疗的 2021 年更新。
Am J Hematol. 2021 Jul 1;96(7):872-888. doi: 10.1002/ajh.26240. Epub 2021 May 31.
4
Two cases of special POEMS syndrome without monoclonal protein expression: a case report and literature review.两例无单克隆蛋白表达的特殊POEMS综合征病例报告及文献复习
J Int Med Res. 2021 Apr;49(4):300060521990967. doi: 10.1177/0300060521990967.
5
Clinical and electrophysiological profiles in early recognition of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes syndrome.早期识别多发性神经病、器官肿大、内分泌病、M 蛋白和皮肤改变综合征的临床和电生理特征。
Chin Med J (Engl). 2019 Jul 20;132(14):1666-1672. doi: 10.1097/CM9.0000000000000318.
6
[How I diagnose and treat POEMS syndrome].[我如何诊断和治疗POEMS综合征]
Zhonghua Xue Ye Xue Za Zhi. 2019 May 14;40(5):368-371. doi: 10.3760/cma.j.issn.0253-2727.2019.05.004.
7
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。
Am J Hematol. 2019 Jul;94(7):812-827. doi: 10.1002/ajh.25495. Epub 2019 May 23.
8
What is the best first-line treatment for POEMS syndrome: autologous transplantation, melphalan and dexamethasone, or lenalidomide and dexamethasone?POEMS 综合征的一线治疗方法哪种最好:自体移植、马法兰和地塞米松,还是来那度胺和地塞米松?
Leukemia. 2019 Apr;33(4):1023-1029. doi: 10.1038/s41375-019-0391-2. Epub 2019 Jan 30.
9
Clinical characteristics and the long-term outcome of patients with atypical POEMS syndrome variant with undetectable monoclonal gammopathy.不伴单克隆丙种球蛋白血症的非典型 POEMS 综合征变异患者的临床特征和长期预后。
Ann Hematol. 2019 Mar;98(3):735-743. doi: 10.1007/s00277-018-03589-4. Epub 2019 Jan 5.
10
Efficacy and Long-Term Outcomes of Autologous Stem Cell Transplantation in POEMS Syndrome: A Nationwide Survey in Japan.POEMS 综合征患者自体造血干细胞移植的疗效和长期预后:日本全国性调查。
Biol Blood Marrow Transplant. 2018 Jun;24(6):1180-1186. doi: 10.1016/j.bbmt.2018.01.026. Epub 2018 Feb 1.