全反式维甲酸联合化疗治疗小儿急性早幼粒细胞白血病

目的观察全反式维甲酸（ATRA）联合化疗治疗小儿急性早幼粒细胞白血病（APL）的疗效。方法22例初治患儿用ATRA诱导治疗；当患儿获完全缓解（CR）后，给予DA方案或IDA方案或HA方案或AA方案巩固治疗3个疗程；以后再用ATRA、化疗交替巩固治疗36个月。结果2例在诱导治疗前死于弥散性血管内凝血（DIC）、颅内出血；22例获CR，CR率100％（20／20）。1、3、5年无病生存（DFS）率分别为100％（20／20）、93．3％（14／15）、84．7％（11／13）。ATRA常见毒副作用依次为皮肤和口唇干燥、头痛、恶心、呕吐、肝功能损害及维甲酸综合征。结论ATRA联合化疗治疗小儿APLCR率高、远期疗效好；在诱导治疗前，DIC、颅内出血仍是APL患儿死亡的主要原因；ATRA毒副作用可耐受。

Outcome of childhood acute promyelocytic leukemia treated with all-trans-retinoic acid and chemotherapy

Objective To determine the results of treatment combining all-trans-retinoic acid(ATRA)in childhood acute promyelocytic leukemia(APL).Methods 22 children with newly diagnosed APL received induction therapy with ATRA followed by 3 courses of consolidation chemotherapy:daunorubicin,idarubicin,homoharringtonine or aclacinomycin plus cytosine arabinoside.A maintenance therapy was then administered with ATRA and these reigems for 36 months.Results Early deaths from diffuse intravazcular clotting and intracranial hemorrhage occurred in two patients.The other children achieved a complete remission(CR).By June 2007,the estimated disease-free survival rates at 1,3 and 5 years were 100%,93.3% and 84.7%;respectively.The side effects of ATRA were xerosis eutis and xerocheilia,headaches,nausea and vomiting,hepatic function lesion and ATRA syndrome.Conclusion Remission induction therapy with ATRA is effective and safe for newly diagnosed childhood APL.The maintenance therapy combined chemotherapy with ATRA can improve the long-term effects of APL patients.The main causes of death in APL children is diffuse intravascular clotting and intracranial hemorrhage.The side effects of ATRA can be tolerated.