Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.
Department of Diagnostic Pathology, Kanazawa University Hospital, Kanazawa, Japan.
Pediatr Hematol Oncol. 2023 May;40(4):363-370. doi: 10.1080/08880018.2022.2109784. Epub 2022 Sep 20.
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.
伴神经母细胞瘤的眼阵挛-肌阵挛综合征（OMS-NB）是一种难治性副肿瘤综合征，常遗留神经后遗症，其详细发病机制仍难以捉摸。我们遇到一例接受免疫抑制治疗的伴 OMS-NB 的儿科患者，该患者表现为抗谷氨酸受体 δ2 抗体和脑脊液（CSF）中 B 细胞增多，肿瘤组织中存在多个富含 B 细胞的淋巴滤泡。无偏倚 B 细胞受体库分析显示，CSF 和肿瘤组织中均存在相同的 B 细胞克隆，该克隆占优势。这些相同的 B 细胞克隆可能有助于 OMS-NB 的发病机制。我们的结果可能有助于为 OMS-NB 建立基于发病机制的治疗策略。
