EB病毒相关性嗜血细胞性淋巴组织细胞增生症患儿的临床特点及预后分析  

Retrospective analysis of clinical characteristics and factors affecting prognosis of children with Epstein-Barr virus associated hemophagocytic lymphohistiocytosis

作　　者：任伟[1] 李小琳[1] 刘玉玲[1] 张莉[1] 

机构地区：[1]南方医科大学附属中山市博爱医院儿科,广东中山528403

出　　处：《检验医学与临床》2014年第15期2068-2069,2072,共3页Laboratory Medicine and Clinic

摘　　要：目的探讨儿童EB病毒相关性嗜血细胞性淋巴组织细胞增生症(EBV-HLH)的临床特点及预后危险因素。方法回顾性分析本院2003年4月至2013年6月收治的22例EBV-HLH患者的临床特征,包括年龄、性别、临床表现、实验室资料及预后,采用Logistic回归分析影响预后的因素。结果 EBV-HLH的发病年龄为3个月至14岁,高峰在1~3岁,所有患儿均有发热,铁蛋白升高,至少有外周血两系减低。伴有肝肿大19例(86.3%),脾肿大16例(72.7%),淋巴结肿大14例(63.6%)。血生化检查肝功能损害,凝血功能异常;骨髓中找到嗜血细胞13例(59.1%),所有病例血清中EBV-DNA检测阳性,病毒载量copy在1.60×103 copy/mL^3.29×107copy/mL之间;22例中死亡9例,好转或治愈9例,失访4例。铁蛋白(SF)大于2 000ng/mL,乳酸脱氢酶(LDH)大于2 000U/L与EBV-HLH的预后呈显著相关性,差异有统计学意义(P<0.05)。结论 EBV-HLH在儿童中病死率高、预后差。SF、乳酸脱氢酶(LDH)水平是影响其预后的主要危险因素。Objective To evaluate the clinical features of Epstein-Barr virus associated hemophagocytic lym-phohistiocytosis（ EBV-HLH） in children and to explore its prognosis and risk factors .Methods A retrospective study was performed on 22 pediatric patient with EBV-HLH who were admitted to our hospital between Apr .2003 to Jun .2008 .All patients medical records were reviewed and analyzed .For each patient ,demographic ,clinical laboratory and outcome information was collected .Logistic regression analysis was performed to determine possible factors affecting prognosis .Results The age of onset ranged from 2 month to 14 years and most of the patients were between l and 3 years of age .All patients exhibited fever ,elevation of serum ferritin and cytopenia ≥2 cell lines .Most of the patient presented with hepatomegaly （86 .3% ） ,splenomegaly （72 .7% ） and lymphadenopathy （63 .6% ） .Laboratory tests showed liver dysfunction ,coagulation disorders .Hemophagocytosis was found in bow narrow of 59 .1% pa-tients .All cases detected EBV-DNA in serum ,Virus load between 1 .60 × 10^3 copy/mL -3 .29 × 10^7 copy/mL patients .Nine patients died ,9 patients recovered ,while 4 patients missed during follow-up .Multivariate Logistic regression indicated that level of SF 〉 2 000 ng/mL and LDH 〉 2 000 U/L significantly predicted an increased risk of death .Conclusion EBV-HLH is a severe disease with a high fatality rate that occurs mainly after EBV reactivation . Early initiation of chemotherapy and timely diagnosis significantly improve survival rate .Level of SF and LDH were the main risk factors of affect the prognosis .

关 键 词：淋巴组织细胞增生症 嗜血细胞 儿童 临床特点 危险因素 

分 类 号：R725.511[医药卫生—儿科]