Department of Neurology, Yale School of Medicine, 15 York Street, New Haven, CT, 06510, USA.
Department of Neurology, Heidelberg University, Heidelberg, Germany.
J Neurooncol. 2020 Aug;149(1):153-159. doi: 10.1007/s11060-020-03583-9. Epub 2020 Jul 18.
Recent studies suggest a relatively high prevalence of autoimmune disorders (AD) among primary CNS lymphoma (PCNSL) patients, however, the literature is limited to case reports. To gain a better understanding of AD-PCNSL we reviewed and analyzed all cases described in the literature.
We searched the MEDLINE database using the search terms 'central nervous system lymphoma' or 'CNS lymphoma' along with AD-related terms. We selected 39 records for qualitative synthesis of data and identified 50 AD-PCNSL. Clinical, imaging and outcome data were collected. Overall survival (OS) was analyzed with the Kaplan-Meier method. Univariate and multivariate analyses were performed using log rank test and Cox proportional hazard model.
Most common AD were systemic lupus erythematosus (24%), multiple sclerosis (16%), and myasthenia gravis (14%). All patients had received immunosuppressants for their AD. Median interval from AD until PCNSL diagnosis was 108 months (range: 11-420). Male-to-female ratio was 0.42 and AD-PCNSL was diagnosed at a median age of 57 years (range: 2-88). On imaging lesions typically localized to the hemispheres (65%) and displayed peripheral enhancement (74%). Pathological evaluation revealed diffuse large-B-cell lymphoma (DLBCL) subtype (80%) and Epstein-Barr virus positivity (75%) in most AD-PCNSL. Median OS was 31 months. Age > 60 years (p = 0.014) was identified as a significant prognostic factor.
AD requiring immunosuppression appear over-represented in the population of PCNSL patients. Aggressive polychemotherapy can accomplish long term OS in AD-PCNSL comparable to immunocompetent patients. Age > 60 may serve as a prognostic factor.
最近的研究表明，原发性中枢神经系统淋巴瘤（PCNSL）患者中自身免疫性疾病（AD）的患病率相对较高，但是文献仅限于病例报告。为了更好地了解 AD-PCNSL，我们回顾和分析了文献中描述的所有病例。
我们使用“中枢神经系统淋巴瘤”或“CNS 淋巴瘤”以及 AD 相关术语在 MEDLINE 数据库中进行搜索。我们选择了 39 个记录进行数据定性综合，并确定了 50 例 AD-PCNSL。收集了临床、影像和结局数据。使用 Kaplan-Meier 方法分析总生存期（OS）。使用对数秩检验和 Cox 比例风险模型进行单变量和多变量分析。
最常见的 AD 是系统性红斑狼疮（24%）、多发性硬化症（16%）和重症肌无力（14%）。所有患者均因 AD 接受免疫抑制剂治疗。从 AD 到 PCNSL 诊断的中位间隔时间为 108 个月（范围：11-420）。男女比例为 0.42，AD-PCNSL 的诊断中位年龄为 57 岁（范围：2-88）。影像学上病变通常位于半球（65%），呈外周强化（74%）。病理评估显示大多数 AD-PCNSL 为弥漫性大 B 细胞淋巴瘤（DLBCL）亚型（80%）和 Epstein-Barr 病毒阳性（75%）。中位 OS 为 31 个月。年龄＞60 岁（p=0.014）被确定为显著的预后因素。
需要免疫抑制的 AD 在 PCNSL 患者人群中似乎过度表达。积极的联合化疗可以使 AD-PCNSL 患者获得与免疫功能正常患者相当的长期 OS。年龄＞60 岁可能是一个预后因素。
