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48例原发性中枢神经系统淋巴瘤的回顾性研究

[Retrospective study of 48 cases of primary central nervous system lymphoma].

作者信息

Alessandro Lucas, Pastor Rueda José M, Villalonga Juan F, Bruno Verónica A, Carpani Federico, Blaquier Juan B, Tognarelli Sofía, Varela Francisco J, Muggeri Alejandro

机构信息

Servicio de Neurología, Instituto de Investigaciones Neurológicas Raúl Carrea (FLENI), Buenos Aires, Argentina. E-mail:

Servicio de Neurología, Instituto de Investigaciones Neurológicas Raúl Carrea (FLENI), Buenos Aires, Argentina.

出版信息

Medicina (B Aires). 2017;77(1):17-23.

Abstract

Primary central nervous system lymphoma (PCNSL) is an infrequent form of non-Hodgkin lymphoma restricted to the CNS. More than 90% are type B and mainly affect patients aged 50-70 years. Immunodeficiency is the most important risk factor. The aim of our study was to evaluate the immune status, clinical presentation and findings in complementary studies of PCNSL patients. A retrospective analysis of 48 cases treated in our center between January 1992 and May 2015 was performed. Median age at diagnosis was 61 years (range 25-84); with male predominance (2.1:1). Forty one cases (85%) were immunocompetent patients. Brain MRI findings showed parenchymal involvement in 45 cases (94%), 43% with frontal lobe and 35% basal ganglia, 4% had meningeal involvement and 2% had ophthalmic involvement at diagnosis. Fifty-five percent had restricted signal on diffusion weighted imaging and contrast enhancement was found in 89%. Pyramidal syndrome was the main initial clinical manifestation (56%). There were abnormal findings in 62% of CSF samples, but in only 11.1% positive cytology results were detected. The most frequent type was diffuse large B-cell lymphoma (83%), being B-cell type the most common form between them (96%). In our series PCNSL was more frequent in immunocompetent elderly male subjects. At initial evaluation, clinical manifestations and MRI findings were variable. The initial suspicion of this entity would allow an early diagnosis, avoiding empirical treatments that may confuse or delay diagnosis.

摘要

原发性中枢神经系统淋巴瘤(PCNSL)是一种罕见的非霍奇金淋巴瘤,局限于中枢神经系统。超过90%为B型,主要影响50 - 70岁的患者。免疫缺陷是最重要的危险因素。我们研究的目的是评估PCNSL患者在补充研究中的免疫状态、临床表现和检查结果。对1992年1月至2015年5月在我们中心治疗的48例患者进行了回顾性分析。诊断时的中位年龄为61岁(范围25 - 84岁);男性占优势(2.1:1)。41例(85%)为免疫功能正常的患者。脑部MRI检查结果显示,45例(94%)有实质受累,其中43%累及额叶,35%累及基底节,4%在诊断时有脑膜受累,2%有眼部受累。55%在扩散加权成像上有受限信号,89%有对比增强。锥体综合征是主要的初始临床表现(56%)。62%的脑脊液样本有异常发现,但仅11.1%检测到阳性细胞学结果。最常见的类型是弥漫性大B细胞淋巴瘤(83%),其中B细胞型是最常见的形式(96%)。在我们的系列研究中,PCNSL在免疫功能正常的老年男性受试者中更为常见。在初始评估时,临床表现和MRI检查结果各不相同。对该疾病的初步怀疑有助于早期诊断,避免可能混淆或延误诊断的经验性治疗。

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