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

IF 1.2 4区 医学 Q4 HEMATOLOGY Pediatric Hematology and Oncology Pub Date : 2023-05-01 DOI:10.1080/08880018.2022.2109784
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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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.

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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
CiteScore
2.60
自引率
5.90%
发文量
71
审稿时长
6-12 weeks
期刊介绍: PHO: Pediatric Hematology and Oncology covers all aspects of research and patient management within the area of blood disorders and malignant diseases of childhood. Our goal is to make PHO: Pediatric Hematology and Oncology the premier journal for the international community of clinicians and scientists who together aim to define optimal therapeutic strategies for children and young adults with cancer and blood disorders. The journal supports articles that address research in diverse clinical settings, exceptional case studies/series that add novel insights into pathogenesis and/or clinical care, and reviews highlighting discoveries and challenges emerging from consortia and conferences. Clinical studies as well as basic and translational research reports regarding cancer pathogenesis, genetics, molecular diagnostics, pharmacology, stem cells, molecular targeting, cellular and immune therapies and transplantation are of interest. Papers with a focus on supportive care, late effects and on related ethical, legal, psychological, social, cultural, or historical aspects of these fields are also appreciated. Reviews on important developments in the field are welcome. Articles from scientists and clinicians across the international community of Pediatric Hematology and Oncology are considered for publication. The journal is not dependent on or connected with any organization or society. All submissions undergo rigorous peer review prior to publication. Our Editorial Board includes experts in Pediatric Hematology and Oncology representing a wide range of academic and geographic diversity.
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