室管膜型原发性中枢神经系统淋巴瘤四例:临床表现及脑脊液细胞学分析

Primary central nervous system lymphoma with ependymal involvement:analysis of clinical manifestations and cerebrospinal fluid cytological features of 4 cases

目的分析室管膜型原发性中枢神经系统淋巴瘤(PCNSL)的临床及影像学特点,探讨脑脊液细胞学检测方法在诊断中的价值。方法回顾4例室管膜型PCNSL患者临床诊断与治疗经过,分析其临床特点、影像学改变、脑脊液细胞学和免疫细胞化学染色结果。结果 4例患者平均发病年龄为44岁,发病至就诊时间13 d～5个月。临床表现为头痛、脑膜刺激征,分别伴有脊神经根征(2例)、多组脑神经麻痹(1例)和偏瘫(1例);病程中有间断低热(3例)。其中2例于发病2和4个月时死于脑疝。腰椎穿刺检查脑脊液压力(2例)、白细胞计数(4例)和蛋白定量(4例)升高,葡萄糖降低(3例)。4例患者脑脊液细胞学检测均发现淋巴瘤细胞或异形淋巴细胞,免疫细胞化学染色大多数细胞呈现B细胞标记物阳性。头部MRI增强扫描第三和第四脑室、侧脑室壁异常强化,合并脑室周围强化病灶(2例)和鞍区病变(1例)。PET扫描和骨髓穿刺检查未发现颅外或脊髓受累证据。结论脑脊液细胞学和免疫细胞化学检测是诊断室管膜型PCNSL的重要方法。室管膜及脑室周围病变应考虑PCNSL的可能,尤其MRI增强扫描发现结节样强化者更应提高警惕。

Objective To analyse clinical and cerebrospinal fluid cytological features of primary central nervous system lymphoma （PCNSL） with ependymal involvement and investigate the diagnostic value of cerebrospinal fluid cytology. Methods Clinical and radiological features of 4 cases of PCNSL were reviewed retrospectively. The findings in conventional cytology and immunocytochemistry of cerebrospinal fluid were analysed. Results All of the patients suffered from headache. Meningeal irritation signs were positive in all the 4 cases. Other clinical features were as follow： radiculoneuropathy in 2 cases, multiple cranial nerves paralysis in one case, bemiplegia in one case, and intermittent low-grade fever in 3 cases. Two cases died of cerebral hernia 2-4 months later after the onset. Lumber puncture was performed in 3 cases and intracranial hypertension was found in 2 cases. One case received ventricular drainage. Cerebrospinal fluid examination showed lymphocyte pleocytosis （4 cases） with elevated protein （4 cases） and lower glucose level （3 eases）. The examination of cerebrospinal fluid found lymphocyte pleocytosis with remarkable atypical lymphoma cells in all of the cases. Among 3 patients received eerebrospinal fluid immunocytochemieal studies, the lymphoeytes were positive for CD19, CD20, CD79a and implied a B- lymphocyte source. Brain magnetic resonance imaging （MRI） showed irregular enhancement of the ventricular wall with nodular periventricular and septal enhancement in part of the 4 eases and pituitary enhancement in one ease. Two patients were diagnosed via biopsy of the brain, which showed non- Hodgkin＇s lymphoma. The other two were diagnosed through cytology and immunocytoehemieal studies of cerebrospinal fluid. One case received positron emission tomography （PET） examination, which showed no evidence of extracranial involvement. Bone marrow biopsy in other patients were normal too. Conclusion Cytological and immunocytochemieal studies of cerebrospinal fluid specimen should be stressed in the diagnosis of PCNSL. This report also emphasizes the importance of clinical and radiological findings in the diagnosis of this disease.