82例朗格罕斯细胞组织细胞增生症临床分析

Analysis of 82 children with langerhans cell histiocytosis

目的分析82例郎格汉斯细胞组织细胞增生症(LCH)患儿的治疗效果,旨在了解LCH的长期预后。方法收集2001年6月-2011年5月我院收治的初治LCH患儿,其中2001年6月-2005年12月发病者按改良DAL-HX 83/90方案治疗,设为DAL-HX 83/90组,共27例;2006年1月-2011年5月发病者按LCH-Ⅲ方案治疗,设为LCH-Ⅲ组,共55例。所有患儿均按国际组织细胞协会疗效标准评价疗效。结果 82例患儿化疗结束后总有效率为87%,其中DAL-HX83/90组治疗总有效率为82%,LCH-Ⅲ组治疗总有效率为89%,两者比较差异无显著性(P=0.54)。82例患儿5年无事件生存率(EFS)为82%,5年总生存率(OS)为95%。DAL-HX 83/90组和LCH-Ⅲ组患儿复发率分别为22%和16%(χ2=0.12,P=0.73),3年EFS分别为78%和84%(χ2=0.14,P=0.71)。结论采用改良DAL-HX 83/90方案和LCH-Ⅲ方案的近、远期疗效相仿,均可获得与国外研究近似的有效率和生存率。对有高危脏器累及、复发难治LCH患儿的治疗,仍是目前临床难题。

Objective This study was designed to improve the long-term prognosis of Langerhans cell histiocytosis （LCH） by analyzing the therapeutic effect of eighty-two patients with LCH treated in our hospital.Methods Between June 2001 and May 2011,eighty-two patients with newly diagnosed LCH were enrolled in this study.The patients were classified into 2 groups by different protocols.From June 2001 to December 2005,twenty-seven cases were enrolled in the modified DAL-HX 83/90 protocol group.From January 2006 to May 2011,fifty-five cases were enrolled in theLCH-Ⅲ protocol group.The short and long-term outcomes of all cases were evaluated by the efficacy criteria of International Study Group of the Histiocyte Society.Results The total effective rate of all cases was 86.6% when the protocol was finished.In the modified DAL-HX 83/90 group,the total effective rate was 81.5%,while that in the LCH-Ⅲgroup was 89.1%.There was no significant difference between the two groups（P =0.54）.The 5-year event-free survival （EFS） and 5-year overall survival （OS） for all cases were 81.7% and 95.1%,respectively.In the modified DAL-HX 83/90 group,the relapse rate was 22.2%,while that in the LCH-Ⅲ group was 16.4%.There was nosignificant difference between the two groups （x2 =0.12,P=0.73）.In the modified DAL-HX 83/90 group,the 3-year EFS was 77.8%,while that in the LCH-Ⅲ group was 83.6%.No significant difference was seen between the groups （x2 =0.14,P =0.71）.Conclusion The short and long-term outcomes of the modified DAL-HX 83/90 protocol and the LCH-Ⅲ protocol was comparable.Both of these two protocols could obtain the similar effective and survival rate with the study abroad.How to cure the high-risk organ involvement,recurrent and refractory LCH was the difficult problem for clinical treatment in recent years.