MGUS的神经学表现。

IF 2.9 3区 教育学 Q1 EDUCATION, SCIENTIFIC DISCIPLINES Hematology. American Society of Hematology. Education Program Pub Date : 2024-12-06 DOI:10.1182/hematology.2024000665
M Teresa Cibeira, Luis Gerardo Rodríguez-Lobato, Aida Alejaldre, Carlos Fernández de Larrea
{"title":"MGUS的神经学表现。","authors":"M Teresa Cibeira, Luis Gerardo Rodríguez-Lobato, Aida Alejaldre, Carlos Fernández de Larrea","doi":"10.1182/hematology.2024000665","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":12973,"journal":{"name":"Hematology. American Society of Hematology. Education Program","volume":"2024 1","pages":"499-504"},"PeriodicalIF":2.9000,"publicationDate":"2024-12-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11665721/pdf/","citationCount":"0","resultStr":"{\"title\":\"Neurological manifestations of MGUS.\",\"authors\":\"M Teresa Cibeira, Luis Gerardo Rodríguez-Lobato, Aida Alejaldre, Carlos Fernández de Larrea\",\"doi\":\"10.1182/hematology.2024000665\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":12973,\"journal\":{\"name\":\"Hematology. American Society of Hematology. Education Program\",\"volume\":\"2024 1\",\"pages\":\"499-504\"},\"PeriodicalIF\":2.9000,\"publicationDate\":\"2024-12-06\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11665721/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Hematology. American Society of Hematology. Education Program\",\"FirstCategoryId\":\"95\",\"ListUrlMain\":\"https://doi.org/10.1182/hematology.2024000665\",\"RegionNum\":3,\"RegionCategory\":\"教育学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"EDUCATION, SCIENTIFIC DISCIPLINES\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Hematology. American Society of Hematology. Education Program","FirstCategoryId":"95","ListUrlMain":"https://doi.org/10.1182/hematology.2024000665","RegionNum":3,"RegionCategory":"教育学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"EDUCATION, SCIENTIFIC DISCIPLINES","Score":null,"Total":0}
引用次数: 0

摘要

未确定意义单克隆伽玛病(MGUS)是一种非常普遍的疾病,其特征是骨髓浆细胞或淋巴浆细胞克隆小(小于10%),产生少量单克隆副蛋白,无相关器官损害。大多数MGUS患者无限期地表现出良性行为,但有些进展为明显的恶性肿瘤,而另一些患者在单克隆蛋白没有增加的情况下发生器官损害,导致所谓的临床意义MG (MGCS)。这一概念包括不同的疾病,取决于所涉及的器官,其中,从诊断和治疗的角度来看,神经学意义的MG (MGNS)构成了一个真正的挑战。由于MGNS的异质临床表现和通常缺乏诊断性活检,诊断尤其困难。另一方面,治疗的复杂性在于缺乏标准化的治疗方案和神经损伤的普遍不可逆性。针对影响周围神经系统的MGUS的神经学表现,我们从日常实践中描述了3例具有代表性的病例,并讨论了诊断到治疗的不同方面,强调需要神经学家和血液学家密切合作进行多学科管理。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
Neurological manifestations of MGUS.

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.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
Hematology. American Society of Hematology. Education Program
Hematology. American Society of Hematology. Education Program EDUCATION, SCIENTIFIC DISCIPLINES-HEMATOLOGY
CiteScore
4.70
自引率
3.30%
发文量
0
期刊介绍: Hematology, the ASH Education Program, is published annually by the American Society of Hematology (ASH) in one volume per year.
期刊最新文献
Novel conditioning and prophylaxis regimens for relapse prevention. On the horizon: upcoming new agents for the management of ITP. Patient-reported outcomes after CAR T-cell therapy in patients with hematological malignancies. The POD24 challenge: where do we go from here for early progressors? Givosiran: a targeted treatment for acute intermittent porphyria.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1