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中枢神经系统原发性套细胞淋巴瘤 被引量:1

Primary central nervous system mantle cell lymphoma
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摘要 目的报告1例中枢神经系统原发性套细胞淋巴瘤患者的临床、病理以及影像学表现.以提高对该病的认识。方法回顾性分析患者临床表现、影像学特征、细胞学与组织病理学表现以及治疗情况,并对脑脊液细胞和异常脑组织进行免疫组织化学染色和基因重排检测。结果临床主要表现为阵发性头晕、渐进性加重的行走不稳伴恶心、呕吐。头部MRI检查脑实质呈多发异常信号.无强化和占位效应。脑脊液细胞学检查呈现小至中等大小的淋巴细胞,细胞核形态欠规则,可见核分裂象:组织病理学显示胶质纤维背景中散在分布有淋巴细胞,可见血管周聚集现象及软脑膜浸润,肿瘤细胞染色质深染,少部分细胞可见小核仁,核分裂象少见;肿瘤细胞表达CD20、CD5及细胞周期素D1等标志物。基因重排检测免疫球蛋白重链呈单克隆性。采用以大剂量甲氨蝶呤为主的化疗方案,同时神经鞘内注射利妥昔单抗注射液,治疗2d后患者出现昏迷,因家属拒绝抢救而出院。结论中枢神经系统原发性套细胞淋巴瘤为临床罕见淋巴瘤,其组织学表现与发生在颅外组织者不同,影像学表现亦有别于其他类型的中枢神经系统原发性淋巴瘤,临床治疗方案尚有待进一步探讨。 Objective To introduce the clinical, pathological and imaging manifestations of one case with primary central nervous system (CNS) mantle cell lymphoma in order to improve the understanding to this kind of disease. Methods Retrospective study was performed to analyse the clinical manifestations, imaging characteristics, cytologic and histopathologic findings, and treatment information of the patient. Immunohistochemical staining and gene rearrangement detecting were done to the cells of cerebrospinal fluid (CSF) and the abnormal brain tissue. Results The main clinical manifestations were paroxysmal dizziness, progressively developing walking unsteadiness with nausea and emesis. The brain MRI showed multiple abnormal signals without reinforcement and space occupying effect. Many small to medium size lymphocytes with irregular and cleaved nucleus were found by CSF cytologic examination. In histopathologic examination, lymphocytes with anachromasis chromatin and few mitosis in nuclues diffusely distributed in the abnormal brain tissue, lesser cells with small nucleolus, focal cells gathered around vessels and some cells involved meningina. The tumor cells were positive for CD20, CD5 and cyclin D1, monoclonicity of IgH was detected by PCR gene arrangement, Chemotherapy with predominant methotrexate (MTX) and intrathecal injection with rituximab injection (mabthera) for the patient were performed and two days later, the patient became coma and discharged due to patient's family member who rejected doctors' salvage. Conclusion Primary central nervous system mantle cell lymphoma is a very rare kind of lymphoma, which is different from the tissue framework outside brain in the same type lymphoma and different from MRI in other type of primary central nervous system lymphoma and the treatment is not yet definite.
出处 《中国现代神经疾病杂志》 CAS 2008年第1期43-47,共5页 Chinese Journal of Contemporary Neurology and Neurosurgery
关键词 淋巴瘤 B细胞 脑肿瘤 磁共振成像 病理学 Lymphoma, B cell Brain neoplasms Magnetic resonance imaging Pathology
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