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目的探讨噬血细胞综合征(HLH)-2004方案(简称HLH-2004方案)治疗儿童HLH的疗效及影响疗效的相关因素。方法回顾性分析华中科技大学同济医学院附属同济医院2005年1月至2009年12月符合国际组织细胞协会HLH-2004诊断标准的28例患儿的临床特点,根据国际组织细胞协会2004年提出的疗效标准,观察其对HLH-2004方案治疗反应及转归,并分析可能影响HLH患儿疗效的相关因素。结果 28例HLH患儿接受HLH-2004方案治疗,随访时间2周至22个月,获得完全缓解或治愈17例(60.7%);出院失访9例(32.1%),其中获临床有效或好转出院失访3例,未获缓解自动出院失访6例;死亡2例(7.2%)。结论 HLH-2004是诊断和治疗儿童HLH的有效方案,早期积极治疗原发病及采用HLH-2004免疫化疗方案,有效地控制病情,提高患儿治愈率及生存率。

The therapeutic effect of HLH-2004 protocol for hemophagocytic lymphohistiocytosis in children

Objective Explore the therapeutic effect of HLH-2004 protocol announced by Histiocyte Society in children with hemophagocytic lymphohistiocytosis(HLH) and the related factors affecting curative effect.Methods Cases of children of our hospital in January 2005-December 2009 based on diagnostic criteria of HLH-2004 were retrospectively analyzed.According to criterion of therapeutic effect of HLH-2004,observe their clinical symptoms and reaction to the treatment,and analyze related factors which possibly affect the curative effect of HLH in children.Results Totally 28 cases of HLH patients received HLH-2004 therapy,and follow-up time ranged from 2 weeks to 22 months ;17 patients(60.7%) achieved complete remission or cure,patients lost to follow-up in 9 cases(32.1%),among which 3 cases have been clinically effective or improved discharge lost,while 6 cases lost to follow-up has not been ease ;2 cases(7.2%) died.Conclusion Early positive treatment of the primary disease and the use of HLH-2004 immune chemotherapy in children with HLH effectively control the disease,improving the cure rate and survival rate in children.