原发中枢神经系统自然杀伤/T细胞脑膜淋巴瘤一例临床分析

Clinical study of a rare case of primary central nervous system natural killer/T cell meningeal lymphoma

目的 探讨原发中枢神经系统自然杀伤/T（NK/T）细胞脑膜淋巴瘤的临床特点和诊断方法.方法 报道1例原发中枢神经系统NK/T细胞脑膜淋巴瘤患者,并结合文献总结其临床特征.结果 患者青年男性,急性起病,表现为视物成双、头痛、呕吐,口角歪斜,短期内出现全身疼痛、四肢无力,腰穿白细胞和蛋白显著升高,脑脊液细胞学曙红亚甲基蓝Ⅱ和迈-格-姬染色可见大量异型淋巴细胞,核异型性明显,可见核分裂相;免疫细胞化学染色可见多数淋巴样细胞Ki-67阳性,提示为增殖活跃的淋巴瘤.脑脊液流式细胞学：97.98%的细胞表达细胞膜型CD3、CD7、CD56、CD2和CD5,部分表达CDs,免疫分型为恶性NK/T细胞;头颅MRI＋强化提示双侧三叉神经增粗,软脑膜强化,增粗的三叉神经轻度强化;骨髓检查未见异常,胸腹CT及强化未见异常.依据患者的病史、体格检查、临床检测和疾病经过,诊断为原发中枢神经系统NK/T细胞脑膜淋巴瘤.结论 原发中枢神经系统NK/T细胞脑膜淋巴瘤是罕见的中枢神经系统淋巴瘤,具有特殊的免疫分型,临床表现为恶性进展的高颅压、脑神经和脊神经受累,脑脊液细胞学和流式细胞分型对确定诊断起关键作用,放化疗效果欠佳,预后差.

Objective To explore the clinical features and diagnostic method of primary natural killer（ NK）/T cell meningeal lymphoma. Methods An unusual case of a 19-year-old male with primary NK/T cell meningeal lymphoma was reported. His clinical presentation and laboratory findings were discussed. The related literatures have been reviewed. Results The patient presented with diplopia,headache, vomiting and facial drooping at the onset, followed by progressive pain and weakness of the four limbs. Cerebrospinal fluid showed significant increase in pressure, leukocytes number, levels of protein,normal glucose and adenosine deaminase, negative tuberculosis antibody and sterile staining. In cerebrospinal fluid cytological analysis, May-Grunwald-Gimsa staining showed large number of atypical lymphocytes with irregular nucleus and nuclear fission, Ki-67 immunostaining showed extensive proliferative activity of the lymphoid cells. Flow cytometric immunophenotypic analysis of cerebrospinal fluid indicated 97. 98 percent of cells expressed surface CD3, CD7, CD56, CD2, CD5, and partially expressed CD8. This was a rare immunophenotype for NK/T-cell. Cranial MRI with gadolinium showed thickening of the trigeminal nerve with slight enhancement and diffuse leptomeningeal enhancement. CT of the chest and abdomen and bone marrow biopsies were negative. He was diagnosed as primary NK/T cell meningeal lymphoma based on the clinical features and related examination. Conclusions Primary NK/T cell meningeal lymphoma is a rare type of primary central nervous lymphomas which has special immunophenotype. The clinical features include progressive raised intra-cranial pressure, multiple cranial and spinal nerve involvements. Cerebrospinal fluid cytological analysis and flow cytometric immunophenotypic analysis are key work-up for diagnosis. It has poor response to chemotherapy and radiotherapy.