HLH-2004方案治疗83例EB病毒相关噬血淋巴组织细胞增生症患儿疗效分析  

Clinical features and outcome analysis of 83 childhood Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with HLH-2004 protocol

作　　者：肖莉[1] 宪莹[1] 戴碧涛[1] 苏庸春[1] 肖剑文[1] 郑启城[1] 赵晓东[1] 于洁[1] 

机构地区：[1]重庆医科大学附属儿童医院血液肿瘤科教育部儿童发育疾病研究重点实验室,400014

出　　处：《中华血液学杂志》2011年第10期668-672,共5页Chinese Journal of Hematology

基　　金：重庆市教委基金（KJ080311）

摘　　要：目的了解儿童EB病毒相关噬血淋巴组织细胞增生症（Epstein—Barrvirus—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〈30g／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。Objective To investigate the clinical features of Epstein-Barr virus-related hemophago- cytic 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-2004 protocol from 83 pediatric patients with EBV-HLH enrolled from January 2006 to December 2009 in our hosip- ital were analyzed retrospectively. Univariate and multivariate COX regression analysis were used to indentify statistically significant prognostic factors. Results （1）Among the 83 patients, 45 were males and 38 were fe- males. 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 ） %. （2）The 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 !n bone marrow at diagnosis of EBV-HLH. （3）COX 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.

关 键 词：淋巴组织增殖性疾病 治疗结果 儿童 HLH-2004方案 EB病毒 

分 类 号：R72[医药卫生—儿科]