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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。
Objective To investigate the therapeutic effect of HLH-2004 (HLH-2004) on children with HLH and its related factors. Methods The clinical features of 28 children who met HLH-2004 diagnostic criteria from January 2005 to December 2009 at Tongji Hospital affiliated to Tongji Medical College of Huazhong University of Science and Technology were retrospectively analyzed. According to the International Organization of Cell Societies’ 2004 proposal The therapeutic effect of HLH-2004 regimen was observed, and the related factors that may affect the efficacy of HLH were analyzed. Results 28 children with HLH were treated with HLH-2004 and were followed up for 2 weeks to 22 months. 17 patients (60.7%) were completely relieved or cured, 9 patients (32.1%) were discharged from hospital, 3 cases were lost to follow-up, 6 cases were lost to spontaneous discharge without remission, and 2 died (7.2%). Conclusion HLH-2004 is an effective method for the diagnosis and treatment of children with HLH. Active treatment of primary disease and HLH-2004 immunotherapy and chemotherapy can effectively control the disease and improve the cure rate and survival rate of children.