目的观察国际组织细胞协会制定的LCH-Ⅲ方案的治疗效果,提高儿童郎格汉斯细胞组织细胞增生症(LCH)的疗效。方法 36例LCH患儿均经临床、实验室检查及活组织病理检查确诊,按疾病严重程度分为多系统高危险度(Ⅰ组)、多系统低危险度(Ⅱ组)及单系统多发和局限性特殊部位累及(Ⅲ组)3组,采用改良LCH-Ⅲ方案分别以不同化疗强度治疗,按国际组织细胞协会疗效标准进行评价。结果Ⅰ组14例,Ⅱ组10例,Ⅲ组12例。化疗结束时完全缓解50.00%,好转33.33%。随访33例,复发4例(均为Ⅰ组),其中2例原方案再治疗缓解,2例放弃治疗死亡。另1例缓解期死于感染。目前持续缓解中14例,占38.89%。其中Ⅰ组2例(14.29%),Ⅱ组8例(80.00%),Ⅲ组4例(33.33%)。结论改良LCH-Ⅲ方案疗效显著,多系统低危险度组(Ⅱ组)预后较好,多系统高危险度(Ⅰ组)患儿中有缓解后复发,复发患儿原方案治疗仍可能获缓解。 Objective To observe the therapeutic effect of LCH-Ⅲ regimen developed by the International Association of Cell Organisms and to improve the therapeutic effect of Langerhans cell histiocytosis (LCH) in children. Methods Thirty - six cases of LCH children were diagnosed by clinical examination, laboratory examination and biopsy. According to the severity of the disease, they were divided into high risk group (group Ⅰ), low risk of multiple system (group Ⅱ) Limitations of special parts involved (group Ⅲ) three groups, using modified LCH-Ⅲ program were treated with different intensity of chemotherapy, according to the criteria of the International Organization of the. Results Group Ⅰ 14 cases, Ⅱ group 10 cases, Ⅲ group 12 cases. The complete remission 50.00% at the end of chemotherapy, improved 33.33%. Follow-up was performed in 33 cases and in 4 cases (all in group Ⅰ). Two of the two patients were re-treated and re-treated, and two were given up for treatment. Another case of remission died of infection. At present, 14 cases continued to ease, accounting for 38.89%. There were 2 cases in group Ⅰ (14.29%), 8 cases in group Ⅱ (80.00%) and 4 cases (33.33%) in group Ⅲ. Conclusions The modified LCH-Ⅲ regimen has a significant therapeutic effect. The prognosis of the multi-system low-risk group (group Ⅱ) is better than that of the multi-system low-risk group (group Ⅰ) .