噬血细胞综合征患者桥脑病变MRI表现及病因探讨  

MRI characteristics and etiology of pontine lesions in patients with hemophagocytic syndrome

作　　者：乔鹏岗 程旭 孙如镜 张诗雨 吴林[1] 脱厚珍[1] 杨正汉[1] QIAO Peng-gang;CHENG Xu;SUN Ru-jing;ZHANG Shi-yu;WU Lin;TUO Hou-zhen;YANG Zheng-han(Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China)

机构地区：[1]首都医科大学附属北京友谊医院,北京100050

出　　处：《中国临床医学影像杂志》2023年第5期309-313,共5页Journal of China Clinic Medical Imaging

基　　金：北京市医院管理中心临床医学发展专项经费(ZYLX202101);北京市重大疫情防治重点专科项目(京卫医〔2021〕135号)。

摘　　要：目的:探讨噬血细胞综合征(HLH)患者桥脑病变MRI特征及病因。资料与方法:回顾性分析2019年6月—2022年6月北京友谊医院18例HLH患者桥脑病变的MRI特征及临床资料,MRI分析包括病变位置、平扫及增强信号特征、是否有桥脑外病灶、复查变化,并与临床症状、血液生化结果、脑脊液生化结果进行对照分析。结果:18例患者中16例无中枢神经系统症状;15例(83.3%)病变检出前曾发生电解质紊乱(低钠或低钾),14例行脑脊液检查者3例(21.4%)合并脑脊液生化异常;MRI表现为桥脑中央部病灶,T1WI低信号,T2WI及FLAIR高信号,12例(66.7%)表现为DWI高信号但ADC值不低,3例(16.7%)表现为DWI高信号且ADC值减低,6例(33.3%)检出桥脑外病灶;电解质紊乱纠正及鞘内注射化疗药物后,桥脑病变变化不一,与临床预后无确切关联。结论:当HLH患者发生桥脑中央病变时,可能的原因为桥脑中央髓鞘溶解(CPM)或HLH中枢神经系统(CNS-HLH)受累,临床上需关注电解质变化情况,警惕CPM的发生及进展,同时结合脑脊液生化检查判断有无CNS-HLH并进行针对性的治疗。Objective:To investigate the MRI characteristics and etiology of pontine lesions in patients with hemophago-cytic syndrome(HLH).Materials and Methods:MRI features and clinical data of 18 HLH patients with pontine lesions in Beijing Friendship Hospital from June 2019 to June 2022 were retrospectively analyzed.The MRI analysis included the loca-tion of pontine lesions,signal characteristics of plain and enhanced MRI scan,presence or absence of extrapontine lesions,the reexamination changes,and the results were compared with clinical symptoms,blood biochemical results and cerebrospinal flu-id biochemical result.Result:Among the 18 patients,16 had no central nervous system symptoms.Fifteen cases(83.3%)had electrolyte disorder(low sodium or low potassium)before the detection of the lesion.Among the 14 patients who underwent cerebrospinal fluid examination,3(21.4%)were complicated with biochemical abnormalities of cerebrospinal fluid.The lesions all located in the center of pontine,the signal of MRI showed hypointense on T1WI,hyperintense on T 2WI and FLAIR.Twelve cases(66.7%)showed hyperintense on DWI without low ADC value,and 3 cases(16.7%)showed hyperintense on DWI with low ADC value.Six cases(33.3%)showed extrapontine lesions.After correction of electrolyte disorder and intrathecal in-jection of chemotherapeutic drugs,the changes of pontine lesions were different,and there was no definite correlation with clinical prognosis.Conclusion:When central pontine lesions occur in HLH patients,the possible causes are central pontine myelinolysis(CPM)or HLH central nervous system(CNS-HLH)involvement.Clinicians should pay attention to electrolyte changes,be alert to the occurrence and progress of CPM,and judge whether CNS-HLH exists and carry out targeted treat-ment in combination with biochemical examination of cerebrospinal fluid.

关 键 词：淋巴组织细胞增多症 噬血细胞性 脑疾病 磁共振成像 

分 类 号：R472[医药卫生—护理学] R445.2[医药卫生—临床医学]