Presence of identical B-cell clone in both cerebrospinal fluid and tumor tissue in a patient with opsoclonus-myoclonus syndrome associated with neuroblastoma.

IF 16.4 1区 化学 Q1 CHEMISTRY, MULTIDISCIPLINARY
Kazuhiro Noguchi, Yasuhiro Ikawa, Mika Takenaka, Yuta Sakai, Toshihiro Fujiki, Rie Kuroda, Hiroko Ikeda, Satoko Nakada, Kozo Nomura, Seisho Sakai, Masaki Fukuda, Raita Araki, Yukitoshi Takahashi, Taizo Wada
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引用次数: 0

Abstract

Opsoclonus-myoclonus syndrome associated with neuroblastoma (OMS-NB) is a refractory paraneoplastic syndrome which often remain neurological sequelae, and detailed pathogenesis has remained elusive. We encountered a pediatric patient with OMS-NB treated by immunosuppressed therapy who showed anti-glutamate receptor δ2 antibody and increased B-cells in cerebrospinal fluid (CSF), and multiple lymphoid follicles containing abundant Bcells in tumor tissue. Unbiased B-cell receptor repertoire analysis revealed identical B-cell clone was identified as the dominant clone in both CSF and tumor tissue. These identical B-cell clone may contribute to the pathogenesis of OMS-NB. Our results could facilitate the establishment of pathogenesis-based treatment strategies for OMS-NB.

在脑脊液和肿瘤组织中存在相同的b细胞克隆与神经母细胞瘤相关的阵挛-肌阵挛综合征患者。
神经母细胞瘤伴眼阵挛-肌阵挛综合征(OMS-NB)是一种顽固性副肿瘤综合征,常伴有神经系统后遗症,其具体发病机制尚不清楚。我们遇到一名接受免疫抑制治疗的小儿OMS-NB患者,其脑脊液(CSF)中出现抗谷氨酸受体δ2抗体和b细胞升高,肿瘤组织中出现多个含有丰富b细胞的淋巴滤泡。无偏b细胞受体库分析显示,在脑脊液和肿瘤组织中发现相同的b细胞克隆为优势克隆。这些相同的b细胞克隆可能参与了OMS-NB的发病机制。我们的研究结果有助于建立基于发病机制的OMS-NB治疗策略。
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来源期刊
Accounts of Chemical Research
Accounts of Chemical Research 化学-化学综合
CiteScore
31.40
自引率
1.10%
发文量
312
审稿时长
2 months
期刊介绍: Accounts of Chemical Research presents short, concise and critical articles offering easy-to-read overviews of basic research and applications in all areas of chemistry and biochemistry. These short reviews focus on research from the author’s own laboratory and are designed to teach the reader about a research project. In addition, Accounts of Chemical Research publishes commentaries that give an informed opinion on a current research problem. Special Issues online are devoted to a single topic of unusual activity and significance. Accounts of Chemical Research replaces the traditional article abstract with an article "Conspectus." These entries synopsize the research affording the reader a closer look at the content and significance of an article. Through this provision of a more detailed description of the article contents, the Conspectus enhances the article's discoverability by search engines and the exposure for the research.
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