儿童嗜血细胞性淋巴组织细胞增生症预后危险因素分析  

Analysis of prognostic risk factors in children with hemophagocytic lymphohistiocytosis

作　　者：李玉川[1] 翟晓文[1] 王宏胜[1] 钱晓文[1] 苗慧[1] 朱晓华[1] 俞懿[1] 李军[1] 陆凤娟[1] 

机构地区：[1]复旦大学附属儿科医院血液科,上海201102

出　　处：《临床血液学杂志》2015年第6期933-938,共6页Journal of Clinical Hematology

摘　　要：目的:通过分析嗜血细胞性淋巴组织细胞增生症(HLH)患儿的临床表现、诊断标准、治疗以及预后情况,探讨影响HLH预后的危险因素。方法:回顾性分析65例HLH患儿的临床特征、实验室检查结果、治疗方案及预后。结果:65例HLH患儿中,男女之比为1.71∶1.00,发病中位年龄26个月。病因以EB病毒相关性HLH最为常见,共35例(53.85%),风湿免疫性疾病相关HLH 2例(3.08%),原发性HLH 2例(3.08%)。临床表现以发热65例(100%),肝脏肿大63例(96.92%),脾脏肿大54例(83.08%)最为常见。实验室检查以起病时肝功能异常最为明显,铁蛋白升高65例(100%),外周血两系以上细胞减少55例(84.62%),纤维蛋白原降低55例(84.62%),高甘油三酯血症(TG≥3.0mmol/L)47例(72.31%),骨穿发现嗜血现象39例(60.94%)。单因素分析显示,出血倾向、低白蛋白血症及总胆红素升高是影响HLH预后的危险因素;多因素Logistic分析显示,出血倾向及低白蛋白血症是影响HLH预后的独立危险因素。结论:HLH病因复杂,临床表现和实验室检查异常多样,预后凶险,早期诊断和规范治疗十分重要。出血倾向和低白蛋白血症是影响HLH预后的危险因素。Objective：To investigate the clinical characteristics,diagnosis,treatment and outcomes of hemophagocytic lymphohistiocytosis（HLH）in children and to explore the risk factors influencing mortality.Method：A retrospective study was carried out on 65 pediatric patients with HLH.All their medical records were reviewed and analyzed.For each patient,demographic,clinical and laboratory data and outcome information were collected.Result：Among the 65 HLH patients,the male/female ratio was 1.71∶1.00,the median age was 26 months.The underlying cause of HLH was Epstein-Barr virus infection in 35patients（53.85%）,autoimmune disorder in 2patients（3.08%）and primary HLH in 2patients（3.08%）.In this study,HLH was clinically characterized by prolonged fever（65patients,100%）,hepatomegaly（63patients,96.92%）,splenomegaly（54patients,83.08%）.Laboratory data indicated that liver dysfunction was the most prominent feature,and the others were hyperferritinemia（65patients,100%）,cytopenias affecting at least two of three lineages in the peripheral blood（55patients,84.62%）,hypofibrinogenemia（55patients,84.62%）,hypertriglyceridemia（47patients,72.31%）,hemophagocytosis in bone marrow（39patients,60.94%）.Univariate analysis showed that the hemorrhagic tendency and level of albumin and total bilirubin were prognostic factors.Multivariate analysis revealed that hemorrhagic tendency and hypoalbuminaemia were independent prognostic factors.Conclusion：There are various underlying pathogenesis and clinical features for HLH.HLH is a severe disease with a high mortality rate.Early diagnosis and treatment is essential.Hemorrhagic tendency and hypoalbuminaemia are the death-associated risk factors.

关 键 词：嗜血细胞性淋巴组织细胞增生症 儿童 危险因素 预后 

分 类 号：R557.4[医药卫生—血液循环系统疾病]