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原发性中枢神经系统淋巴瘤与瘤样多发性硬化症:一项诊断挑战

Primary CNS Lymphoma vs. Tumefactive Multiple Sclerosis: A Diagnostic Challenge.

作者信息

Naeem Sameen Bin, Niazi Farheen, Baig Atif, Sadiq Hina, Sattar Mubbasher

机构信息

Department of Medicine, PAEC General Hospital, Islamabad.

Department of Neurology, PAEC General Hospital, Islamabad.

出版信息

J Coll Physicians Surg Pak. 2018 Jan;28(1):66-68. doi: 10.29271/jcpsp.2018.01.66.

DOI:10.29271/jcpsp.2018.01.66
PMID:29290197
Abstract

Primary CNS (central nervous system) lymphoma is a rare condition with the incidence of less than 1% of all non-Hodgkin lymphomas (NHLs) and approximately 2% of all primary brain tumours. Diagnosis can be challenging and necessitates brain biopsy for definitive diagnosis. A 41-year male presented with history of impaired cognition, facial asymmetry, visual impairment and left sided body weakness. MRI brain demonstrated multiple enhancing lesions with one larger lesion in right basal ganglia with surrounding oedema and mass effect. These findings suggested the differential diagnoses of tumefactive multiple sclerosis (MS), primary CNS lymphoma (PCNSL) and tuberculosis. The patient had normal CT chest, abdomen and pelvis, normal CSF examination and cytology, negative CSF oligoclonal bands (OCBs) and negative HIV screening. It was impossible to differentiate between tumefactive MS and PCNSL without undertaking brain biopsy. Diffuse large B cell lymphoma (DLBCL) was the final diagnosis. Diagnosing PCNSL can be challenging and brain biopsy should not be delayed for definitive diagnosis and targeted treatment.

摘要

原发性中枢神经系统(CNS)淋巴瘤是一种罕见疾病,其发病率在所有非霍奇金淋巴瘤(NHL)中不到1%,在所有原发性脑肿瘤中约为2%。诊断可能具有挑战性,需要进行脑活检以明确诊断。一名41岁男性患者,有认知障碍、面部不对称、视力障碍和左侧身体无力的病史。脑部MRI显示多个强化病灶,右侧基底节有一个较大病灶,伴有周围水肿和占位效应。这些发现提示需鉴别诊断瘤样多发性硬化(MS)、原发性中枢神经系统淋巴瘤(PCNSL)和结核病。患者胸部、腹部和骨盆CT正常,脑脊液检查及细胞学检查正常,脑脊液寡克隆带(OCB)阴性,HIV筛查阴性。不进行脑活检就无法区分瘤样MS和PCNSL。最终诊断为弥漫性大B细胞淋巴瘤(DLBCL)。诊断PCNSL可能具有挑战性,不应延迟脑活检以进行明确诊断和靶向治疗。

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