副肿瘤性脊髓炎伴发瓦尔登斯特伦巨球蛋白血症,对伊布替尼-venetoclax治疗有反应:病例报告

Q3 Neuroscience
Sathya Narasimhan , Kristin M. Galetta , Jorge J. Castillo , Shamik Bhattacharyya
{"title":"副肿瘤性脊髓炎伴发瓦尔登斯特伦巨球蛋白血症,对伊布替尼-venetoclax治疗有反应:病例报告","authors":"Sathya Narasimhan ,&nbsp;Kristin M. Galetta ,&nbsp;Jorge J. Castillo ,&nbsp;Shamik Bhattacharyya","doi":"10.1016/j.ensci.2024.100527","DOIUrl":null,"url":null,"abstract":"<div><p>Waldenström macroglobulinemia (WM) is a B-cell lymphoproliferative malignancy characterized by IgM paraproteinemia and presence of lymphoplasmacytic cells in the bone marrow. Isolated longitudinally extensive transverse myelitis (LETM) is a rare manifestation of WM. We report a rare case of paraneoplastic LETM in a 68-year-old male with treatment-naïve WM (MYD88 L265P mutation in bone marrow aspirate), who responded to ibrutinib and venetoclax therapy. Our patient presented with a two-month history of unsteadiness, tingling, and numbness in both hands and feet, that progressed to bilateral leg and arm weakness. Based on radiographic findings, a diagnosis of paraneoplastic LETM was made and he was treated acutely with IV methylprednisolone followed by a quick oral prednisone taper. However, he subsequently relapsed and symptomatically worsened while on rituximab therapy. Accounting for worsening anemia, our patient was enrolled in a Phase II trial evaluating the effects of ibrutinib-venetoclax therapy in treatment naïve WM. After three months of study therapy, he had a complete response of myelopathy symptoms and MRI lesions. Our observation of sustained disease response in this patient may support a role for concurrent BTK and BCL2 inhibition in paraneoplastic myelitis associated with B-cell lymphoproliferative disorders. However, this observation needs to be validated in larger cohort studies and potentially in clinical trials if further data are supportive.</p></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"37 ","pages":"Article 100527"},"PeriodicalIF":0.0000,"publicationDate":"2024-09-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405650224000340/pdfft?md5=43afc1ecba5891cbfde9596c785398c2&pid=1-s2.0-S2405650224000340-main.pdf","citationCount":"0","resultStr":"{\"title\":\"Paraneoplastic myelitis associated with Waldenström macroglobulinemia responsive to treatment with ibrutinib – venetoclax: A case report\",\"authors\":\"Sathya Narasimhan ,&nbsp;Kristin M. Galetta ,&nbsp;Jorge J. Castillo ,&nbsp;Shamik Bhattacharyya\",\"doi\":\"10.1016/j.ensci.2024.100527\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><p>Waldenström macroglobulinemia (WM) is a B-cell lymphoproliferative malignancy characterized by IgM paraproteinemia and presence of lymphoplasmacytic cells in the bone marrow. Isolated longitudinally extensive transverse myelitis (LETM) is a rare manifestation of WM. We report a rare case of paraneoplastic LETM in a 68-year-old male with treatment-naïve WM (MYD88 L265P mutation in bone marrow aspirate), who responded to ibrutinib and venetoclax therapy. Our patient presented with a two-month history of unsteadiness, tingling, and numbness in both hands and feet, that progressed to bilateral leg and arm weakness. Based on radiographic findings, a diagnosis of paraneoplastic LETM was made and he was treated acutely with IV methylprednisolone followed by a quick oral prednisone taper. However, he subsequently relapsed and symptomatically worsened while on rituximab therapy. Accounting for worsening anemia, our patient was enrolled in a Phase II trial evaluating the effects of ibrutinib-venetoclax therapy in treatment naïve WM. After three months of study therapy, he had a complete response of myelopathy symptoms and MRI lesions. Our observation of sustained disease response in this patient may support a role for concurrent BTK and BCL2 inhibition in paraneoplastic myelitis associated with B-cell lymphoproliferative disorders. However, this observation needs to be validated in larger cohort studies and potentially in clinical trials if further data are supportive.</p></div>\",\"PeriodicalId\":37974,\"journal\":{\"name\":\"eNeurologicalSci\",\"volume\":\"37 \",\"pages\":\"Article 100527\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-09-07\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.sciencedirect.com/science/article/pii/S2405650224000340/pdfft?md5=43afc1ecba5891cbfde9596c785398c2&pid=1-s2.0-S2405650224000340-main.pdf\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"eNeurologicalSci\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S2405650224000340\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"Neuroscience\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"eNeurologicalSci","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2405650224000340","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"Neuroscience","Score":null,"Total":0}
引用次数: 0

摘要

瓦尔登斯特伦巨球蛋白血症(WM)是一种 B 细胞淋巴增生性恶性肿瘤,其特征是 IgM 副蛋白血症和骨髓中存在淋巴浆细胞。孤立性纵向广泛横贯性脊髓炎(LETM)是 WM 的一种罕见表现。我们报告了一例罕见的副肿瘤性LETM病例,患者是一名68岁的男性,患有治疗无效的WM(骨髓抽吸中发现MYD88 L265P突变),对伊布替尼和venetoclax治疗有反应。患者两个月前出现双手和双脚不稳、刺痛和麻木,随后发展为双侧腿部和手臂无力。根据影像学检查结果,诊断为副肿瘤性 LETM,并对他进行了静脉甲基强的松龙急性治疗,随后快速减量口服强的松。然而,他的病情随后复发,并在接受利妥昔单抗治疗期间出现症状恶化。考虑到贫血的恶化,我们的患者被纳入了一项II期试验,评估伊布替尼-文尼考克斯疗法对治疗新药WM的影响。经过三个月的研究治疗后,他的脊髓病症状和磁共振成像病变均已完全反应。我们在这名患者身上观察到的持续疾病反应可能支持同时抑制BTK和BCL2在与B细胞淋巴细胞增生性疾病相关的副肿瘤性骨髓炎中发挥作用。不过,这一观察结果还需要在更大规模的队列研究中进行验证,如果有进一步的数据支持,还可能在临床试验中进行验证。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Paraneoplastic myelitis associated with Waldenström macroglobulinemia responsive to treatment with ibrutinib – venetoclax: A case report

Waldenström macroglobulinemia (WM) is a B-cell lymphoproliferative malignancy characterized by IgM paraproteinemia and presence of lymphoplasmacytic cells in the bone marrow. Isolated longitudinally extensive transverse myelitis (LETM) is a rare manifestation of WM. We report a rare case of paraneoplastic LETM in a 68-year-old male with treatment-naïve WM (MYD88 L265P mutation in bone marrow aspirate), who responded to ibrutinib and venetoclax therapy. Our patient presented with a two-month history of unsteadiness, tingling, and numbness in both hands and feet, that progressed to bilateral leg and arm weakness. Based on radiographic findings, a diagnosis of paraneoplastic LETM was made and he was treated acutely with IV methylprednisolone followed by a quick oral prednisone taper. However, he subsequently relapsed and symptomatically worsened while on rituximab therapy. Accounting for worsening anemia, our patient was enrolled in a Phase II trial evaluating the effects of ibrutinib-venetoclax therapy in treatment naïve WM. After three months of study therapy, he had a complete response of myelopathy symptoms and MRI lesions. Our observation of sustained disease response in this patient may support a role for concurrent BTK and BCL2 inhibition in paraneoplastic myelitis associated with B-cell lymphoproliferative disorders. However, this observation needs to be validated in larger cohort studies and potentially in clinical trials if further data are supportive.

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
eNeurologicalSci
eNeurologicalSci Neuroscience-Neurology
CiteScore
3.50
自引率
0.00%
发文量
45
审稿时长
62 days
期刊介绍: eNeurologicalSci provides a medium for the prompt publication of original articles in neurology and neuroscience from around the world. eNS places special emphasis on articles that: 1) provide guidance to clinicians around the world (Best Practices, Global Neurology); 2) report cutting-edge science related to neurology (Basic and Translational Sciences); 3) educate readers about relevant and practical clinical outcomes in neurology (Outcomes Research); and 4) summarize or editorialize the current state of the literature (Reviews, Commentaries, and Editorials). eNS accepts most types of manuscripts for consideration including original research papers, short communications, reviews, book reviews, letters to the Editor, opinions and editorials. Topics considered will be from neurology-related fields that are of interest to practicing physicians around the world. Examples include neuromuscular diseases, demyelination, atrophies, dementia, neoplasms, infections, epilepsies, disturbances of consciousness, stroke and cerebral circulation, growth and development, plasticity and intermediary metabolism. The fields covered may include neuroanatomy, neurochemistry, neuroendocrinology, neuroepidemiology, neurogenetics, neuroimmunology, neuroophthalmology, neuropathology, neuropharmacology, neurophysiology, neuropsychology, neuroradiology, neurosurgery, neurooncology, neurotoxicology, restorative neurology, and tropical neurology.
×
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
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
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 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信