表现为多发脑内微出血的中枢神经系统血管内淋巴瘤1例临床分析并文献复习  

Clinical analysis of a case of central nervous system intravascular lymphoma with multiple intracerebral microbleeds and literature

作　　者：刘颖[1] 贾国勇[1] 李怡[1] 赵玉英[1] 王翠兰[1] 焉传祝[1] Liu Ying;Jia Guoyong;Li Yi;Zhao Yuying;Wang Cuilan;Yan Chuanzhu(Department of Neurology,Qilu Hospital of Shandong University,Jinan 250012,China)

机构地区：[1]山东大学齐鲁医院神经内科,济南250012

出　　处：《中华神经科杂志》2022年第2期102-108,共7页Chinese Journal of Neurology

摘　　要：目的探讨中枢神经系统血管内大B细胞淋巴瘤(IVLBCL)的临床病理特点及影像鉴别诊断。方法报道山东大学齐鲁医院神经内科2017年诊断的1例表现为多发脑内微出血的中枢神经系统IVLBCL患者,对其临床和影像学资料、组织学及免疫组织化学标记结果进行回顾性分析,并进行相关文献的复习。结果患者为31岁女性,主要表现为头痛、癫痫发作。头颅磁共振成像示双侧大脑半球及右侧小脑半球脑白质区多发斑片状病灶,T_(1)WI及弥散加权成像呈低信号,T_(2)WI及液体衰减反转恢复序列呈高信号,增强扫描示病灶呈小片状强化。磁敏感加权成像示双侧大脑半球皮质及皮质下多发片状低信号,以皮质下为著。头颅磁共振血管造影未见异常,磁共振静脉造影示左侧横窦发育不全;磁共振波谱示N-乙酰基天冬氨酸峰降低,胆碱峰升高,有倒置的脂质双峰。经激素治疗后,颅内病灶的范围明显缩小。但激素停用1周后再次复发,且颅内病变范围较前增多、增大。病理学检查示脑组织内散在多发的微小出血灶及水肿。脑内及脑膜表面的小血管内可见大量异型的单个核细胞聚集。免疫组织化学检查示CD_(20)、CD_(79α)、B细胞淋巴瘤(BCL)-2、BCL-6、髓细胞增生原癌基因(C-myc)、多发性骨髓瘤原癌基因-1(MUM-1)表达阳性,Ki67增殖指数约70%。Epstein-Barr病毒编码的小mRNA、CD_(3)、CD_(10)、角蛋白、CD_(138)等表达呈阴性,确诊IVLBCL。结论IVLBCL是临床罕见病,病情进展迅速,预后极差,早期诊断和治疗是关键。活体组织检查是诊断的“金标准”,随机皮肤活组织检查或许能帮助早期诊断IVLBCL。对影像学表现为多发脑内微出血的患者来说,鉴别诊断除中枢神经系统血管炎及脑淀粉样血管病等常见病因外,要考虑到IVLBCL的可能。Objective To investigate the clinicopathological features and imaging differential diagnosis of intravascular large B-cell lymphoma(IVLBCL)in the central nervous system(CNS).Methods A case of CNS IVLBCL with multiple intracerebral microbleeds(CMBs)diagnosed in the Department of Neurology,Qilu Hospital of Shandong University in 2017 was reported.The clinical and imaging data,histological and immunohistochemical markers were retrospectively analyzed,and the relevant literature was reviewed.Results The patient was a 31-year-old woman presented with headache and seizures.Cranial magnetic resonance imaging(MRI)showed multifocal lesions involving mainly the cortical and subcortical white matter(bilateral cerebral hemisphere and right cerebellar hemisphere),hyperintense signal on T_(2)-weighted and fluid-attenuated inversion recovery images,with hypointense signal on T_(1)-weigthed and diffusion-weighted images and contrast enhancement in some lesions.The susceptibility weighted imaging revealed multifocal cortical or subcortical hypointense lesions,involving mainly the subcortical white matter.Brain magnetic resonance angiography was normal.Brain magnetic resonance venography showed left side transverse sinus was hypoplastic.Cranial magnetic resonance spectroscopy showed decreased N-acetylaspartylglutamate peak,elevated choline peak and inverted lipid double peak.Her symptoms and the lesions once improved after starting steroid treatment.However,CNS recurrence occurred after 1 week of steroid withdrawal.She underwent the biopsy of the right frontotemporal lobe.The pathological examination showed multiple microscopic hemorrhages and edema scattered in the brain tissue.A large number of heterologous mononuclear cells were aggregated in small blood vessels in the parenchyma and meninges.Immunohistochemical analysis revealed that the tumor was negative for Epstein-Barr virus encoded small RNAs,CD_(3),CD_(10),cytokeratin and CD_(138),and positive for CD_(20),CD_(79α),B-cell lymphoma(BCL)-2,BCL-6,myelocytomatosis oncogene(C

关 键 词：淋巴瘤 B细胞 血管 脑出血 脑内多发微出血 

分 类 号：R743.34[医药卫生—神经病学与精神病学] R739.4[医药卫生—临床医学]