CAJ | 학술논문

目的了解儿童EB病毒相关噬血淋巴组织细胞增生症(Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis,EBV-HLH)的临床特征,研究HLH-2004方案对EBV-HLH的疗效,分析影响EBV-HLH预后的危险因素.方法回顾性分析2006年1月至2009年12月在我院诊治的83例EBV-HLH患儿发病时的临床特征及HLH-2004方案治疗EBV-HLH的疗效,采用单因素和多因素COX模型分析影响EBV-HLH预后的危险因素.结果 ①83例患儿中,男45例,女38例,发病年龄6个月～14岁4个月,44例接受HLH-2004方案治疗,3年总体生存率为(55.8±7.9)％.②EBV-HLH患儿高热、肝脾肿大、外周血两系或三系降低、凝血功能障碍多见；呼吸道症状、咽峡炎、皮疹、神经系统表现较少见；97.3％患儿有铁蛋白升高,大多数患儿有肝脏功能损害和脂质代谢紊乱；骨髓检查示89.0％患儿可见噬血细胞.③贫血程度、白蛋白＜30 g/L、CD4+细胞和CD8+细胞比值异常、NK细胞比例＜3％以及治疗方式是影响EBV-HLH患儿预后的独立危险因素(P值均＜0.05).结论 EBV-HLH病情重,预后差；HLH-2004诊治方案是治疗儿童EBV-HLH的有效方案；单用对症治疗不能挽救EBV-HLH患儿的生命.
목적 료해인동EB병독상관서혈림파조직세포증생증(Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis,EBV-HLH)적림상특정,연구HLH-2004방안대EBV-HLH적료효,분석영향EBV-HLH예후적위험인소.방법 회고성분석2006년1월지2009년12월재아원진치적83례EBV-HLH환인발병시적림상특정급HLH-2004방안치료EBV-HLH적료효,채용단인소화다인소COX모형분석영향EBV-HLH예후적위험인소.결과 ①83례환인중,남45례,녀38례,발병년령6개월～14세4개월,44례접수HLH-2004방안치료,3년총체생존솔위(55.8±7.9)％.②EBV-HLH환인고열、간비종대、외주혈량계혹삼계강저、응혈공능장애다견；호흡도증상、인협염、피진、신경계통표현교소견；97.3％환인유철단백승고,대다수환인유간장공능손해화지질대사문란；골수검사시89.0％환인가견서혈세포.③빈혈정도、백단백＜30 g/L、CD4+세포화CD8+세포비치이상、NK세포비례＜3％이급치료방식시영향EBV-HLH환인예후적독립위험인소(P치균＜0.05).결론 EBV-HLH병정중,예후차；HLH-2004진치방안시치료인동EBV-HLH적유효방안；단용대증치료불능만구EBV-HLH환인적생명.
Objective To investigate the clinical features of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis(EBV-HLH),to analysis the outcome of HLH-2004 protocol,and to explore the prognostic factors in EBV-HLH patients.Methods The clinical features at onset and outcome of HLH-2004protocol from 83 pediatric patients with EBV-HLH enrolled from January 2006 to December 2009 in our hosipital were analyzed retrospectively.Univariate and multivariate COX regression analysis were used to indentify statistically significant prognostic factors.Results ①Among the 83 patients,45 were males and 38 were females.The age of onset ranged from 6 months to 14 years 4 months.44 patients were treated with HLH-2004,and 3-year overall survival(OS)was(55.8 ±7.9)％.②The most common clinical features of EBV-HLH included high fever,cytopenia,hepatosplenomegaly,and coagulopathy; The respiratory symptoms,angina phlogistic,skin rashes,neurologic abnormality were rare.97.3％ of patients showed an elevation of serum ferritin,liver dysfunction and lipid metabolism disorders was found in most of EBV-HLH patients.89.0％ of patient had hemophagocytosis in bone marrow at diagnosis of EBV-HLH.③COX regression analysis revealed that anemia degree,serum albumin ＜ 30 g/L,CD4:CD8 abnormity,NK cell ＜ 3％,treatment protocol were related with the prognosis significantly(P ＜ 0.05).Conclusion EBV-HLH in pediatric patients has severe clinical feature and poor prognosis.HLH-2004 protocol is an effective treatment for patients with EBV-HLH.Symptomatic treatment can't rescue the patients of EBV-HLH.