噬血细胞综合征中枢神经系统受累的MRI特征  

MRI features of central nervous system involvement in hemophagocytic syndrome

作　　者：许克宁 张志杰 张艳艳 姜萌 许建生 安冉 魏璇 XU Ke-ning;ZHANG Zhi-jie;ZHANG Yan-yan(Department of Radiology,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China;Department of Radiology,Zhangjiakou First Hospital,Zhangjiakou Hebei 075000,China)

机构地区：[1]首都医科大学附属北京友谊医院放射科,北京100050 [2]张家口市第一医院放射医学科,河北张家口075000

出　　处：《临床和实验医学杂志》2024年第21期2245-2249,共5页Journal of Clinical and Experimental Medicine

基　　金：国家自然科学基金(编号:82202097)。

摘　　要：目的研究噬血细胞综合征(HPS)患者中枢神经系统(CNS)受累的磁共振成像(MRI)成像特点,为影像医师客观评估HPS脑损伤提供方向,为临床医师进一步对症治疗提供影像学依据。方法回顾性分析2015年1月至2023年12月首都医科大学附属北京友谊医院确诊的15例CNS-HPS患者的MRI影像特点。根据信号特征不同,将病灶分为非出血性病灶及出血性病灶。影像特征包括病变分布部位、大小、数目、形态特点、平扫及增强信号特征、伴随脑萎缩情况、复查病灶的对比变化。结果纳入的15例患者中非出血患者13例,出血性患者2例。所有患者病灶数目共计75个。病灶的分布以额顶叶40个(53.3%)非对称性分布为主,病灶大部分表现为斑片状多灶性分布,35个(46.7%)病灶边界清晰。4例(26.7%)出现异常强化。6例(40.0%)患者同时伴有脑萎缩样改变。共9例患者复查,5例病灶减小,3例病灶范围扩大,1例患者第1次复查病灶增多,第2次复发病灶均消失。结论CNS-HPS脑部MRI表现显示出多样性,部分病灶具有特异性,以脑实质改变为主,其次为脑萎缩样改变。MRI可直观反映该疾病脑组织受累的严重程度,成为临床上诊断CNS-HPS的可视化依据。Objective To study the magnetic resonance imaging(MRI)characteristics of central nervous system(CNS)involvement in patients with hemophagocytic syndrome(HPS),to provide a direction for radiologists to objectively evaluate HPS brain injury,and to provide imaging basis for clinicians to further symptomatic treatment.Methods The MRI characteristics of 15 patients with CNS-HPS diagnosed in the Beijing Friendship Hospital,Capital Medical University from January 2015 to December 2023 were retrospectively analyzed.According to the different signal characteristics,the lesions were divided into non-hemorrhagic lesions and hemorrhagic lesions.The imaging features included the location,size,number,morphological characteristics of lesions,the characteristics of noncontrast and enhanced signals,the concomitant cerebral atrophy,and the contrast changes of the lesions on reexamination.Results Among the 15 cases included,13 cases were non-hemorrhagic patients and 2 cases were hemorrhagic patients.The total number of lesions in all patients were 75.The distribution of lesions was mainly asymmetrical distribution of 40(53.3%)lesions in the frontal and parietal lobes,most of the lesions showed a patchy multifocal distribution,and 35(46.7%)lesions had clear boundaries.Four patients(26.7%)had abnormal enhancement.Six patients(40.0%)had concomitant cerebral atrophy-like changes.A total of 9 patients were re-examined,5 cases had decreased lesions,3 cases had expanded lesions,1 cases had more lesions in the first reexamination,and the second recurrence lesions disappeared.Conclusion The brain MRI manifestations of CNS-HPS showed diversity,and some lesions were specific,mainly with brain parenchymal changes,followed by cerebral atrophy-like changes.MRI can intuitively reflect the severity of brain tissue involvement in this disease,and become a visual basis for clinical diagnosis of CNS-HPS.

关 键 词：噬血细胞综合征 磁共振成像 中枢神经系统 血液病 

分 类 号：R445.2[医药卫生—影像医学与核医学] R747.9[医药卫生—诊断学] R593[医药卫生—临床医学]