HLH伴中枢神经系统累及的患儿临床特征及预后危险因素分析  

Analysis of Clinical Characteristics and Prognostic Risk Factors of HLH Children with Central Nervous System Involvement

作　　者：王义江 杨静[2] 屈文静 王沂霞 李祥 WANG Yi-Jiang;YANG Jin;QU Wen-Jing;WANG Yi-Xia;LI Xiang(Department of Pediatrics,The Second Maternal and Child Health Hospital of Jinan,Jinan 271100,Shandong Province,China;Department of Pediatric Hematology,The Affiliated Hospital of Qingdao University,Qingdao 266003,Shandong Province,China)

机构地区：[1]济南市第二妇幼保健院儿科,山东济南271100 [2]青岛大学附属医院血液儿科,山东青岛266003

出　　处：《中国实验血液学杂志》2020年第4期1381-1385,共5页Journal of Experimental Hematology

摘　　要：目的:探讨噬血细胞淋巴组织细胞增生症(HLH)伴中枢神经系统(CNS)累及患儿临床特征及预后危险因素,为进一步改善患儿预后提供更多参考。方法:回顾性分析本院2006年1月-2016年10月收治HLH伴CNS累及的45例患儿临床资料,记录患儿临床特征,针对可能影响患儿预后危险因素建立Cox风险比例模型进行单因素和多因素分析。结果:45例患儿中男19例,女26例,中位年龄为4.0(1-15.1)岁,其中EBV感染38例(84.44%),CMV感染1例(2.22%),细菌感染3例(6.67%),结缔组织疾病1例(2.22%),无明确病因2例(4.44%)。27例行腰椎穿刺检查后证实脑脊液异常10例(37.04%);45例患儿中头颅影像学检查异常22例(48.89%)。45例患儿随访1年总生存率为46.67%(21/45),随访3年总生存率为44.44%(20/45),中位生存时间为5.0个月。脑脊液异常患儿随访1年总生存率显著低于脑脊液正常患儿(10 vs 17例)(P<0.05)。未行鞘内注射患儿随访1年总生存显著低于行鞘内注射患儿(10 vs 17例)(P<0.05)。单因素分析显示,神经系统症状、脑脊液异常、未行鞘内注射及治疗方式均是影响HLH伴CNS累及的患儿预后危险因素(P<0.05)。以上变量引入Cox风险模型行多因素分析显示,脑脊液异常和未行鞘内注射是HLH伴CNS累及的患儿预后独立危险因素(P<0.05)。结论:HLH伴CNS累及的患儿临床预后相对较差,部分患儿后遗神经系统病变;而脑脊液异常和未行鞘注治疗是导致患儿临床预后不良的独立危险因素。Objective:To investigate the clinical characteristics and prognostic risk factors of HLH children with central nervous system(CNS)involvement so as to provide more reference for further improving the prognosis of HLH children.Methods:The clinical data of 45 HLH children with CNS involvement treated in our hospital from January 2006 to October 2016 were collected and analyzed retrospectively.The clinical characteristics of HLH children with CNS involvement were recorded,moreover the possible factors influencing the prognosis of HLH children with CNS involvement were analyzed using univariate and multivariate analysis through the establishment of Cox risk ratio model.Results:Among 45 HLH children with CNS involvement,male was 19 cases and female was 26 cases.The median age of 4.0 years old(1.0-15.1).The detection showed that EBV found in 38 cases(84.44%),CMV infection in 1 case(2.22%),bacterial infection in 3 cases(6.67%),connection tissue disease in 1 case(2.22%)and indefinite etiology infection in 2 cases(4.44%).After lumbar puncture of 27 HLH children with CNS involvement,10 cases(37.04%)showed cerebrospinal fluid abnormality.In addition,22 cases showed the craniography abnormality.The follow-up results showed that the OS rate of 1 year was 46.67%(21/45),the OS rate of 3 years was 44.44%(20/45);the median survival time was 5.0 months.The OS analysis indicated that 1 years OS rate of diseased children with cerebrospinal fluid abnormality was significantly lower than that of diseased children with cerebrospinal fluid normality(10/45 vs 17/45)(P<0.05),and 1 years OS rate of diseased children who not received intrathecal injection was significantly lower that of diseased children who received intrathecal(10/45 vs 17/45)(P<0.05).The univariate analysis showed that the symptoms of nervous system,abnormal cerebrospinal fluid,absence of intrathecal injection and treatment schedule all were the risk factors affecting the prognosis of HLH children with CNS involvement(P<0.05).The multivariate analysis by Cox risk model sh

关 键 词：噬血细胞淋巴组织细胞增生症 中枢神经系统 临床特征 预后 危险因素 

分 类 号：R557.4[医药卫生—血液循环系统疾病]