原发性中枢神经系统淋巴瘤2例临床病理分析并文献复习

Primary central nervous system lymphoma:a report of 2 cases with clinicopathologic analysis and literature review

目的探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)的临床特点、病理诊断、治疗及预后。方法对2例PCNSL进行免疫组化染色并复习相关国内外文献。结果 2例PCNSL,1例为间变性大细胞型-T细胞来源,另1例为弥漫性大B细胞型,HIV均阴性。PCNSL临床表现无特异性,颅内压增高、精神失常为常见症状,影像检查缺乏特征性改变,免疫表型以B细胞为主(87.5%～98%),大剂量氨甲蝶呤联合全脑放疗缓解率高。结论 PCNSL术前难以诊断,需依赖病理检查确诊,治疗困难,预后差。

Purpose To explore the clinical characteristics,pathological features,therapy and prognosis of primary central nervous system lymphoma（PCNSL）.Methods Report of 2 cases PCNSL and relevant literatures were reviewed.Results Two cases with PCNSL were HIV negative,one with anaplastic large cell lymphoma-T cell origin,the other with diffuse large B-cell lymphoma.Clinical presentations of PCNSL were an increased intracranial pressure and focal neurologic deficits with no specific imaging examination.B-cell lymphoma is the predominant subtype（87.5～98%）.High-dose methotrexate-based chemotherapy with whole brain radiotherapy is efficient for PCNSL.Conclusions PCNSL is an aggressive disease with a poor prognosis and difficult to diagnose before operation.The pathological examination might be the only reliable method to confirm PCNSL.