摘要
目的总结中枢神经系统血管内淋巴瘤(IVL)的临床特点、影像学表现和病理特征。方法报道1例以中枢神经系统症状首发并经脑组织活检诊断的血管内淋巴瘤病,并复习和比较相关文献报道。结果患者,38岁,女性,主要表现为肢体乏力、近事记忆力差、认知功能下降、癫痫发作等。头颅MR显示颅内多发的皮层或皮层下异常信号,呈T1WI低信号,T2WI高信号,FLAIR高信号,增强后呈不规则斑片状、环形强化。脑组织活检病理显示毛细血管内异型细胞,LCA、CD20、CD79α表达阳性,个别细胞呈Ki67阳性,确诊为中枢神经系统IVL。术后患者一般情况恶化,经抢救无效死亡。结论中枢神经系统血管内淋巴瘤病进展凶险,预后很差,提高认识,早期诊断和针对性化疗有助于改善患者的预后。
Objective To summarize the clinical, imaging and pathological characteristics of intravascular lym- phoma of the central nervous system （CNS-IVL）. Methods A case initially presenting with neurological symptoms diag- nosed on brain biopsy was described and the related literature of Intravascular lymphoma was reviewed to analyze the charac- teristics of CNS-IVL. Results The patient was a Chinese woman, aged 38. She presented with weakness of right leg, im- pairment of short-term memory, cognitive impairment, consciousness disturbance, and seizure. Brain magnetic resonance imaging （MRI） showed multiple cortical or subcortical lesions that were hypointense on T1 - weighted images, hyperintense on T2-weight and fluid -attenuated inversion recovery （FLAIR） images, and irregular patchy areas with circular enhanced on enhanced images. Pathologically, it was characterized by proliferation of neoplastic cells within the lumina of small ves- sels. Immunohistochemical staining showed that these cells were LCA, CD20, CD79a positive and some cells were also Ki67 positive. The diagnosis of IVL was confirmed. The patient＇s condition deteriorated after operation and she died seven days later. Conclusions Intravascular lymphoma in the central nervous system can progress very rapidly and has with a very poor prognosis. The early diagnosis and combined chemotherapy are beneficial to the patients with intravascular lym- phoma.
出处
《中国神经精神疾病杂志》
CAS
CSCD
北大核心
2010年第7期414-418,共5页
Chinese Journal of Nervous and Mental Diseases
关键词
血管内淋巴瘤
中枢神经系统
活检
intravascular lymphoma central nervous system biopsy
