中枢神经系统淋巴瘤并发干燥综合征1例报告及文献复习

Central nervous system lymphoma complicated with Sjogren’s syndrome:A case report and literature review

目的:分析中枢神经系统淋巴瘤(CNSL)并发干燥综合征(SS)患者的临床表现及诊疗过程,并探讨其发病机制,为该类罕见疾病的诊断提供参考。方法:收集1例CNSL并发SS患者的临床资料,对其诊断经过进行总结,并结合相关文献进行归纳复习。结果:患者,女性,51岁。因口干、眼干伴恶心和呕吐1月余入院。查体,舌面干,时有躁动,四肢肌力4级,左侧指鼻试验欠稳准,左侧跟膝胫试验欠稳准,昂白征阳性,双侧病理征阳性。实验室检查,抗干燥综合征抗体A(抗SSA抗体)和抗干燥综合征抗体B(抗SSB抗体)阳性。泪液分泌实验,左侧5 mm;角膜染色阳性,唾液腺同位素检查阳性。影像学检查,头颅增强磁共振成像(MRI)示双侧小脑半球内侧、视交叉、垂体柄和松果体可见多发异常强化信号;头颅磁共振波谱(MRS)提示N-乙酰天门冬氨酸(NAA)峰和肌酸(Cr)峰减低,胆碱(Cho)峰升高,NAA/Cr=1.74,Cho/Cr=3.52,Cho/NAA=2.02,并可见乳酸(Lac)峰和脂质(Lip)峰,双侧小脑半球病变单和多体素1H-MRS改变。取活检病理检查提示高级别B细胞淋巴瘤伴出血,临床诊断为CNSL并发SS。出院后于当地医院进行放化疗。结论:CNSL并发SS发病率低,易误诊为中枢神经系统脱髓鞘病变,临床上SS患者出现中枢神经系统症状和体征时应考虑CNSL的可能。

Objective:To analyze the clinical manifestation,diagnosis and treatment process of a patient with central nervous system lymphoma(CNSL)complicated with Sjögren’s syndrome(SS)and explore its pathogenesis,and to provide reference for the diagnosis of this rare disease.Methods:The clinical materials of a patient with CNSL complicated with SS were collected.The diagnosis process was summarized and the relevant literatures were reviewed.Results:The female patient,51 year old,was admitted to the hospital for dry mouth and dry eyes with nausea and vomiting for more than one month.The physical examination results showed the tongue surface was dry,the patient moved restlessly at times,and the limb strength was level 4;the left calcaneal and tibial tests were not stable,and the Romberg’s sign were positive and bilateral pathological signs were positive.The results of laboratory tests showed anti-Sjogren’s syndrome A antibody(anti-SSA antibody)and anti-Sjogren’s syndrome B antibody(anti-SSB antibody)were positive.The results of tear secretion test showed left side 5 mm;the corneal staining was positive,and salivary gland isotope test was positive.The enhanced magnetic resonance imaging(MRI)results showed multiple abnormal enhancement signals in bilateral medial cerebellar hemispheres,optic chiasm,pituitary stalk and pineal gland;the magnetic resonance spectrum(MRS)results of the head revealed that the N-acetyl-L-aspartic acid(NAA)peak and creatine(Cr)peak of L-aspartic acid were decreased,while the choline(Cho)peak was increased,NAA/Cr=1.74,Cho/Cr=3.52,Cho/NAA=2.02;the lactate(Lac)peak and lipid(Lip)peak were also seen,single-voxel 1H-MRS and multi-voxel 1HMRS were found in bilateral cerebellar hemispheres.The pathological examination showed that high-grade B-cell lymphoma with hemorrhage and the clinical diagnosis was CNSL complicated with SS.Radiotherapy and chemotherapy were given after discharge in local hospital.Conclusion:The incidence of CNSL with SS is low,and it is easy to be misdiagnosed as central nervous system demyelinating disease.CNSL should be considered when the symptoms and signs of central nervous system are present in the SS patients.