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一名40岁免疫功能正常患者的爱泼斯坦-巴尔病毒阳性原发性中枢神经系统淋巴瘤

Epstein-Barr Virus-Positive Primary Central Nervous System Lymphoma in a 40-Year-Old Immunocompetent Patient.

作者信息

Hajtovic Sabastian, Liu Cynthia, Diefenbach Catherine M, Placantonakis Dimitris G

机构信息

Neurosurgery, City University of New York (CUNY) School of Medicine, New York, USA.

Pathology, New York University (NYU) Grossman School of Medicine, New York, USA.

出版信息

Cureus. 2021 Jan 17;13(1):e12754. doi: 10.7759/cureus.12754.

Abstract

Epstein-Barr virus-positive (EBV+) primary central nervous system lymphoma (PCNSL) is a clinical entity rarely reported in young immunocompetent patients. Here, we present the case of a 40-year-old female with no history of immunosuppression or immunodeficiency, who presented with a ring-enhancing lesion in the right basal ganglia. The tumor generated significant vasogenic edema and mass effect, causing midline shift, symptoms of increased intracranial pressure, and rapidly progressive neurologic dysfunction. She underwent gross total resection of the tumor through a tubular retractor. Her tumor was of the diffuse large B cell lymphoma (DLBCL) subtype of PCNSL and was positive for EBV. No immunodeficiency or extracranial disease was identified. After adjuvant therapy with high-dose methotrexate, rituximab, and temozolomide, she remains disease-free two years after initial presentation. EBV+ PCNSL, although rare in young immunocompetent adults, poses unique clinical challenges and may require surgical intervention in the acute setting in some cases.

摘要

爱泼斯坦-巴尔病毒阳性(EBV+)原发性中枢神经系统淋巴瘤(PCNSL)是一种在免疫功能正常的年轻患者中很少报道的临床实体。在此,我们报告一例40岁女性病例,该患者无免疫抑制或免疫缺陷病史,表现为右侧基底节区环形强化病灶。肿瘤产生了显著的血管源性水肿和占位效应,导致中线移位、颅内压升高症状及快速进展的神经功能障碍。她通过管状牵开器接受了肿瘤全切术。她的肿瘤为PCNSL的弥漫性大B细胞淋巴瘤(DLBCL)亚型,且EBV呈阳性。未发现免疫缺陷或颅外疾病。在接受大剂量甲氨蝶呤、利妥昔单抗和替莫唑胺辅助治疗后,她在初次就诊两年后仍无疾病复发。EBV+ PCNSL虽然在免疫功能正常的年轻成年人中罕见,但会带来独特的临床挑战,在某些情况下可能需要在急性期进行手术干预。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4575/7886623/c31d35f141cd/cureus-0013-00000012754-i01.jpg

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