CAJ | 학술논문

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-HL H ）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-HL H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-HL H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～3．29×107 copy/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2000 ng/mL ，乳酸脱氢酶（LDH）大于2000 U/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。
목적：탐토인동EB병독상관성기혈세포성림파조직세포증생증（EBV-HL H ）적림상특점급예후위험인소。방법회고성분석본원2003년4월지2013년6월수치적22례EBV-HL H환자적림상특정，포괄년령、성별、림상표현、실험실자료급예후，채용Logistic회귀분석영향예후적인소。결과 EBV-HL H적발병년령위3개월지14세，고봉재1～3세，소유환인균유발열，철단백승고，지소유외주혈량계감저。반유간종대19례（86．3％），비종대16례（72．7％），림파결종대14례（63．6％）。혈생화검사간공능손해，응혈공능이상；골수중조도기혈세포13례（59．1％），소유병례혈청중EBV-DNA검측양성，병독재량copy재1．60×103 copy/mL～3．29×107 copy/mL지간；22례중사망9례，호전혹치유9례，실방4례。철단백（SF）대우2000 ng/mL ，유산탈경매（LDH）대우2000 U/L여EBV-HLH적예후정현저상관성，차이유통계학의의（P＜0．05）。결론 EBV-HLH재인동중병사솔고、예후차。SF、유산탈경매（LDH）수평시영향기예후적주요위험인소。
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 af-fecting 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 × 103 copy/mL -3 .29 × 107 copy/mL pa-tients .Nine patients died ,9 patients recovered ,while 4 patients missed during follow-up .Multivariate Logistic regres-sion 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 .