中国实用儿科杂志

中国实用儿科杂志 ›› 2012, Vol. 27 ›› Issue (06): 436-439.

• 论著 • 上一篇    下一篇

HLH-2004方案治疗儿童噬血细胞综合征
28例疗效分析

詹晓芸，胡    群，刘双又，张柳清，刘爱国   

  1. 华中科技大学同济医学院附属同济医院儿科，武汉    430030
  • 出版日期:2012-06-06 发布日期:2012-05-30

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

  • Online:2012-06-06 Published:2012-05-30

摘要：

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。

关键词: 治疗方案, 噬血细胞综合征, 儿童, HLH-2004方案,  , 疗效

Abstract:

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.

Key words: treatment pragrams, hemophagocytic lymphohistoicytosis, children