Zhao Hui, Ma Miao, Zhang Limin, Zheng Guanghui, Lv Hong, Liu Jie, Li Xiao, Song Bei, Zhang Guojun
Laboratory Diagnosis Center, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100050, China.
Beijing Engineering Research Center of Immunological Reagents Clinical Research, Beijing, 100050, China.
BMC Neurol. 2019 May 7;19(1):90. doi: 10.1186/s12883-019-1317-3.
Primary central nervous system lymphoma (PCNSL) is the most prevalent brain, spinal cord, eyes, and leptomeningeal lymphoma. It is often misdiagnosed due to an unspecific presentation or unavailable biopsy and results in a poor prognosis. Although the craniocerebral imaging examination of PCNSL has some characteristics, it is limited, and atypical cases are especially difficult to identify with intracranial tumours and other diseases. The biopsy, as the gold standard for PCNSL diagnosis, is not eligible for all patients suspected of having PCNSL.
This report documents a woman who presented with a three-month history of numbness and weakness in the right leg. She was treated with drugs at a local hospital for one month. She developed demyelination lesions and her symptoms were aggravated. The patient was admitted to the Department of Nerve Infection and Immunology at Tiantan Hospital. Head magnetic resonance imaging (MRI) enhanced scanning indicated significant inflammatory demyelinating disease, and lymphoma was not excluded. CSF revealed a high protein level and CSF cytology detected abnormal cells, PCNSL was eventually presumed according to positive CSF cytology and cytological detection of the cerebrospinal fluid flow.
PCNSL is a highly invasive tumour. With the development of technologies such as cerebrospinal fluid cytology and flow cytology, CSF analysis has become one of the definite diagnosis methods, and the tumour cell finding in CSF is the only reliable basis for diagnosis. Flow cytometric analysis and gene rearrangement testing also provide objective evidence.
原发性中枢神经系统淋巴瘤（PCNSL）是最常见的脑、脊髓、眼和软脑膜淋巴瘤。由于其表现不具特异性或无法进行活检，常被误诊，导致预后不良。尽管PCNSL的颅脑影像学检查有一些特征，但存在局限性，非典型病例尤其难以与颅内肿瘤及其他疾病相鉴别。活检作为PCNSL诊断的金标准，并非适用于所有疑似PCNSL的患者。
本报告记录了一名女性患者，她有三个月的右腿麻木和无力病史。她在当地医院接受了一个月的药物治疗。她出现了脱髓鞘病变，症状加重。患者被收入天坛医院神经感染与免疫科。头部磁共振成像（MRI）增强扫描显示有明显的炎性脱髓鞘疾病，不排除淋巴瘤。脑脊液显示蛋白水平升高，脑脊液细胞学检查发现异常细胞，最终根据脑脊液细胞学阳性及脑脊液流式细胞学检测推测为PCNSL。
PCNSL是一种高侵袭性肿瘤。随着脑脊液细胞学和流式细胞术等技术的发展，脑脊液分析已成为确诊方法之一，脑脊液中发现肿瘤细胞是唯一可靠的诊断依据。流式细胞术分析和基因重排检测也提供了客观证据。
