背景:免疫相关神经综合征(影响中枢和周围神经系统,以及神经肌肉接头)可以与低度B细胞淋巴瘤相关。
方法:我们与内科和血液学服务合作,对患有其他免疫相关神经病的患者的记录进行了回顾性研究,然后是“神经肌肉疾病和ALS转诊中心”(LaTimone医院,和PaoliCalmettes-Insitute,马赛,法国;日内瓦大学医院,日内瓦瑞士)。临床,生物,进行了免疫学和组织学检查,并收集了数据。
结果:我们确定了12例神经系统综合征和不典型表现/病程的患者。在所有这些患者中发现了多种自身抗体。这促使我们进行彻底的血液学调查,这导致诊断不同类型的低度B细胞淋巴瘤[即淋巴浆细胞分化的边缘区淋巴瘤(n=3),脾边缘区淋巴瘤伴继发性淋巴结侵犯(n=1),未分类的边缘区淋巴瘤(n=8)]。下层淋巴瘤的治疗导致神经系统疾病的改善(n=8)或稳定(n=4)。
结论:免疫相关神经综合征的非典型表现,以及具有不同抗原靶标的抗体的存在应被视为“警告信号”,并引起对潜在的低级别B细胞淋巴瘤所维持的副肿瘤起源的怀疑,应积极寻找和治疗.内科医生之间的密切合作,神经学家和血液学家允许对每个病例进行适当的管理。
BACKGROUND: Immune-related neurological syndromes (affecting both the central and peripheral nervous system, as well as the neuromuscular junction) can associate with low-grade B-cell lymphomas.
METHODS: We conducted a retrospective study on the records of patients with miscellaneous immune-related neuropathies followed by the \"Referral Centre for Neuromuscular Diseases and ALS\" in collaboration with the Services of Internal Medicine and Hematology (La Timone Hospital, and the Paoli Calmettes-Insitute, Marseille, France; Geneva University Hospitals, Geneva, Switzerland). Clinical, biological, immunological and histological work-up was carried out and data collected.
RESULTS: We identified 12 patients with neurological syndromes and atypical presentation/course. In all these patients multiple autoantibodies were found. This prompted us to perform thorough hematologic investigations, that led to the diagnosis of different type of Low-Grade B-Cell lymphomas [i.e. marginal zone lymphomas with lymphoplasmacytic differentiation (n=3), splenic marginal area lymphoma with secondary lymph node invasion (n=1), unclassified marginal area lymphomas (n=8)]. Treatment of the underling lymphoma resulted in an improvement (n=8) or stabilization (n=4) of neurological disease.
CONCLUSIONS: Atypical presentation of immune-related neurological syndromes, as well as the presence of antibodies with different antigenic targets should be regarded as \"warning signs\" and raise the suspicion of a paraneoplastic origin sustained by an underlying low-grade B-cell lymphoma that should be actively sought and treated. Close collaboration between internists, neurologists and hematologists allows for the appropriate management of each case.