• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

意义未明的单克隆丙种球蛋白病的神经系统表现。

Neurological manifestations of MGUS.

作者信息

Cibeira M Teresa, Rodríguez-Lobato Luis Gerardo, Alejaldre Aida, Fernández de Larrea Carlos

机构信息

Hematology Department, Amyloid and Myeloma Unit, Hospital Clínic de Barcelona and Institut d´Investigacions Biomèdiques August Pi I Sunyer, University of Barcelona, Barcelona, Spain.

Neurology Department, Amyloid and Myeloma Unit, Hospital Clínic de Barcelona and Institut d´Investigacions Biomèdiques August Pi I Sunyer, University of Barcelona, Barcelona, Spain.

出版信息

Hematology Am Soc Hematol Educ Program. 2024 Dec 6;2024(1):499-504. doi: 10.1182/hematology.2024000665.

DOI:10.1182/hematology.2024000665
PMID:39644073
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11665721/
Abstract

Monoclonal gammopathy of undetermined significance (MGUS) is a highly prevalent disorder characterized by a small bone marrow plasma cell or lymphoplasmacytic clone (less than 10%) that produces a small amount of monoclonal paraprotein without associated organ damage. Most patients with MGUS display benign behavior indefinitely, but some progress to an overt malignancy, and others develop organ damage despite no increase in monoclonal protein, resulting in the so-called MG of clinical significance (MGCS). This concept includes different disorders depending on the organ involved, and among them, MG of neurological significance (MGNS) constitutes a real challenge from both a diagnostic and therapeutic point of view. Diagnosis is particularly difficult due to MGNS's heterogeneous clinical presentation and common lack of a diagnostic biopsy. On the other hand, the complexity of treatment lies in the lack of standardized regimens and the common irreversibility of neurological damage. Focusing on the neurological manifestations of MGUS affecting the peripheral nervous system, we describe 3 illustrative cases from daily practice and discuss different aspects of diagnosis to treatment, emphasizing the need for multidisciplinary management based on the close collaboration of neurologists and hematologists.

摘要

意义未明的单克隆丙种球蛋白病(MGUS)是一种高度常见的疾病,其特征是骨髓中有一个小的浆细胞或淋巴浆细胞克隆(少于10%),产生少量单克隆副蛋白,且无相关器官损害。大多数MGUS患者长期表现为良性,但有些会进展为明显的恶性肿瘤,还有些患者尽管单克隆蛋白没有增加,但仍出现器官损害,导致所谓的具有临床意义的MG(MGCS)。这一概念包括根据受累器官不同的多种疾病,其中,具有神经学意义的MG(MGNS)从诊断和治疗角度来看都是一项真正的挑战。由于MGNS临床表现异质性且通常缺乏诊断性活检,诊断尤为困难。另一方面,治疗的复杂性在于缺乏标准化方案以及神经损伤通常不可逆转。聚焦于影响周围神经系统的MGUS的神经学表现,我们描述了3例日常临床病例,并讨论了从诊断到治疗的不同方面,强调了基于神经科医生和血液科医生密切合作的多学科管理的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f3df/11665721/46bdf4909111/hem.2024000665_s1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f3df/11665721/46bdf4909111/hem.2024000665_s1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f3df/11665721/46bdf4909111/hem.2024000665_s1.jpg

相似文献

1
Neurological manifestations of MGUS.意义未明的单克隆丙种球蛋白病的神经系统表现。
Hematology Am Soc Hematol Educ Program. 2024 Dec 6;2024(1):499-504. doi: 10.1182/hematology.2024000665.
2
The evaluation and management of monoclonal gammopathy of renal significance and monoclonal gammopathy of neurological significance.肾脏意义单克隆丙种球蛋白血症和神经意义单克隆丙种球蛋白血症的评估和管理。
Am J Hematol. 2021 Jul 1;96(7):846-853. doi: 10.1002/ajh.26155. Epub 2021 Mar 25.
3
Diagnosis and Management of Monoclonal Gammopathy of Undetermined Significance: A Review.意义未明的单克隆丙种球蛋白病的诊断与管理:综述
JAMA Intern Med. 2025 Apr 1;185(4):450-456. doi: 10.1001/jamainternmed.2024.8124.
4
Monoclonal gammopathy of undetermined significance: a consensus statement.意义未明的单克隆丙种球蛋白血症:共识声明。
Br J Haematol. 2010 Jul;150(1):28-38. doi: 10.1111/j.1365-2141.2010.08207.x. Epub 2010 May 9.
5
Monoclonal Gammopathy of Undetermined Significance (MGUS)Monoclonal Gammopathy of Undetermined Significance (MGUS).意义未明的单克隆丙种球蛋白病(MGUS)意义未明的单克隆丙种球蛋白病(MGUS)。
Klin Onkol. 2018 Summer;31(4):270-276. doi: 10.14735/amko2018270.
6
Investigation and management of the monoclonal gammopathy of undetermined significance: A British Society for Haematology Good Practice Paper.意义未明的单克隆丙种球蛋白病的调查与管理:英国血液学学会实践指南
Br J Haematol. 2023 Aug;202(4):734-744. doi: 10.1111/bjh.18866. Epub 2023 May 18.
7
Diagnosis, risk stratification and management of monoclonal gammopathy of undetermined significance and smoldering multiple myeloma.意义未明的单克隆丙种球蛋白病和冒烟型多发性骨髓瘤的诊断、风险分层及管理
Int J Lab Hematol. 2016 May;38 Suppl 1:110-22. doi: 10.1111/ijlh.12504. Epub 2016 May 9.
8
[Monoclonal gammopathy of undetermined significance and asymptomatic multiple myelom in the year 2014 ].2014年意义未明的单克隆丙种球蛋白病及无症状多发性骨髓瘤
Vnitr Lek. 2014 Oct;60(10):861-79.
9
Monoclonal Gammopathy of Undetermined Significance: A Comprehensive Review.意义未明的单克隆丙种球蛋白病:全面综述
Clin Lymphoma Myeloma Leuk. 2023 May;23(5):e195-e212. doi: 10.1016/j.clml.2023.02.004. Epub 2023 Feb 23.
10
Are neurological complications of monoclonal gammopathy of undetermined significance underestimated?意义未明的单克隆丙种球蛋白病的神经并发症是否被低估了?
Oncotarget. 2017 Jan 17;8(3):5081-5091. doi: 10.18632/oncotarget.13861.

引用本文的文献

1
Association of product of platelet and neutrophil count with monoclonal gammopathy of undetermined significance: a cross-sectional analysis of the NHANES.血小板与中性粒细胞计数乘积与意义未明的单克隆丙种球蛋白病的关联:美国国家健康与营养检查调查(NHANES)的横断面分析
Blood Res. 2025 Aug 18;60(1):46. doi: 10.1007/s44313-025-00094-2.
2
Monoclonal Gammopathy of Neurological Significance: A Case Report and Insights on Treatment.具有神经学意义的单克隆丙种球蛋白病:一例报告及治疗见解
Mediterr J Hematol Infect Dis. 2025 May 1;17(1):e2025034. doi: 10.4084/MJHID.2025.034. eCollection 2025.

本文引用的文献

1
Paraproteinemic neuropathies.副蛋白血症性神经病。
Muscle Nerve. 2024 Aug;70(2):173-179. doi: 10.1002/mus.28164. Epub 2024 May 30.
2
A novel cell-based immunofluorescence assay for the detection of autoantibodies to myelin-associated glycoprotein.一种用于检测抗髓鞘相关糖蛋白自身抗体的新型细胞免疫荧光检测方法。
Front Neurol. 2023 Dec 14;14:1289810. doi: 10.3389/fneur.2023.1289810. eCollection 2023.
3
Paraproteinemic Neuropathies.副蛋白血症性神经病。
Continuum (Minneap Minn). 2023 Oct 1;29(5):1492-1513. doi: 10.1212/CON.0000000000001294.
4
POEMS syndrome: Update on diagnosis, risk-stratification, and management.POEMS综合征:诊断、风险分层及管理的最新进展
Am J Hematol. 2023 Dec;98(12):1934-1950. doi: 10.1002/ajh.27081. Epub 2023 Sep 21.
5
Guidelines for non-transplant chemotherapy for treatment of systemic AL amyloidosis: EHA-ISA working group.系统性AL淀粉样变性非移植化疗治疗指南:欧洲血液学协会-国际淀粉样变性协会工作组
Amyloid. 2023 Mar;30(1):3-17. doi: 10.1080/13506129.2022.2093635. Epub 2022 Jul 15.
6
IgM monoclonal gammopathies of clinical significance: diagnosis and management.具有临床意义的 IgM 单克隆丙种球蛋白病:诊断与管理。
Haematologica. 2022 Sep 1;107(9):2037-2050. doi: 10.3324/haematol.2022.280953.
7
The International Consensus Classification of Mature Lymphoid Neoplasms: a report from the Clinical Advisory Committee.成熟淋巴细胞肿瘤国际共识分类:临床咨询委员会报告。
Blood. 2022 Sep 15;140(11):1229-1253. doi: 10.1182/blood.2022015851.
8
From Biology to Treatment of Monoclonal Gammopathies of Neurological Significance.从具有神经学意义的单克隆丙种球蛋白病的生物学机制到治疗方法
Cancers (Basel). 2022 Mar 18;14(6):1562. doi: 10.3390/cancers14061562.
9
Oral and Topical Treatment of Painful Diabetic Polyneuropathy: Practice Guideline Update Summary: Report of the AAN Guideline Subcommittee.《疼痛性糖尿病多发性神经病的口服和局部治疗:实践指南更新概要:AAN 指南小组委员会的报告》。
Neurology. 2022 Jan 4;98(1):31-43. doi: 10.1212/WNL.0000000000013038.
10
Guidelines for high dose chemotherapy and stem cell transplantation for systemic AL amyloidosis: EHA-ISA working group guidelines.系统性淀粉样变高剂量化疗和干细胞移植治疗指南:EHA-ISA 工作组指南。
Amyloid. 2022 Mar;29(1):1-7. doi: 10.1080/13506129.2021.2002841. Epub 2021 Nov 16.