文章摘要
黄 婕,王 楠,吴 鹏,张晓军,方拥军.儿童噬血细胞综合征伴中枢神经系统受累临床及生存分析[J].南京医科大学学报,2020,(8):1176~1180
儿童噬血细胞综合征伴中枢神经系统受累临床及生存分析
Clinical features and outcomes of central nervous system involvement of children with hemophagocytic syndrome
投稿时间:2019-12-25  
DOI:10.7655/NYDXBNS20200816
中文关键词: 噬血细胞综合征  中枢神经系统  儿童
英文关键词: hemophagocytic syndrome  central nervous system  child
基金项目:国家自然科学基金(81670155)
作者单位
黄 婕 南京医科大学附属儿童医院血液肿瘤科江苏 南京 210008 
王 楠 南京医科大学附属儿童医院血液肿瘤科江苏 南京 210008 
吴 鹏 南京医科大学附属儿童医院血液肿瘤科江苏 南京 210008 
张晓军 南京医科大学附属儿童医院放射科江苏 南京 210008 
方拥军 南京医科大学附属儿童医院血液肿瘤科江苏 南京 210008 
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中文摘要:
      目的:了解儿童噬血细胞综合征[又称噬血细胞性淋巴细胞组织细胞增多症(hemophagocytic lymphohistiocytosis,HLH)]伴中枢神经系统(central nervous system,CNS)受累临床特点及预后。方法:回顾性分析2008年11月—2017年12月就诊于南京医科大学附属儿童医院的112例HLH患儿中发生CNS受累的29例患儿的临床表现、脑脊液检查结果、CNS影像学结果、治疗方案及转归资料,进行预后及生存分析。结果:29例CNS?HLH患儿中22例(84.1%)伴有严重的神经精神症状,多表现为抽搐和意识不清。28例进行脑脊液检查和CNS影像学检查,其中18例(62.0%)伴有脑脊液检查异常,9例(32.1%)头颅CT或MRI出现异常信号,17 例(60.7%)出现脑萎缩及脑沟宽深、脑室饱满等表现。112例HLH患儿5年累积生存率为75.9%,生存曲线Log?rank检验提示CNS?HLH总体生存较非CNS受累患儿差(χ2=3.936,P=0.047)。接受HLH?2004/94方案治疗的23例CNS?HLH患儿鞘注组(n=17)总体生存显著优于未鞘注组(n=6)(χ2=6.003,P=0.014)。Cox回归多因素分析提示未进行鞘内注射、血红蛋白≤60 g/L是影响CNS?HLH儿童预后的独立危险因素。结论:CNS?HLH总体预后差,HLH患儿需全面评估CNS受累情况,未接受鞘内注射和重度贫血为CNS?HLH的独立预后因素。
英文摘要:
      Objective:To investigate the clinical characteristics and outcomes of central nervous system(CNS) involvement in pediatric patients with hemophagocytic syndrome,also known as hemophagocytic lymphohistiocytosis(HLH). Methods:A total of 112 pediatric patient who were newly diagnosed with HLH at the Children’s Hospital Affiliated to Nanjing Medical University during November 2008 to December 2017 were retrospectively evaluated for CNS involvement. Neurological symptoms,cerebrospinal fluid(CSF) findings,neuroradiological results and treatment outcome were analyzed. Results:About 84.1% patients(22/29)with CNS?HLH in our study had neurologic manifestation. The most two common symptoms were seizures and disturbance of consciousness. CSF analysis and neuroradiological studies were performed in 28 patients. The results showed that 18 patients(62.0%)had CSF abnormalities,9 patients(32.1%)presented nodular or diffuse signal abnormalities,while 17 patients(60.7%)had cerebral atrophy,mild ventriculomegaly or other signs of chronic changes. Five?year cumulative survival rate of 112 patients with HLH was 75.9%. Patients with CNS involvement showed poor overall survival compared to those without CNS involvement(χ2=3.936,P=0.047). Overall survival of CNS?HLH who did not receive intrathecal therapy was inferior to those who received intrathecal therapy(χ2=6.003,P=0.014). Cox multivariate analysis showed that absence of intrathecal therapy and severe anemia(Hb≤60 g/L)at diagnosis were independent risk factors in pediatric CNS?HLH. Conclusion:CNS involvement is associated with poor prognosis in pediatric patients with HLH. Children with HLH require overall assessment of neurological involvement and appropriate therapy. Lack of intrathecal therapy and severe anemia are related to poor prognosis.
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