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目的探讨儿童急性早幼粒细胞性白血病(APL)的临床特点及其治疗。方法选取2002年6月~2007年3月我院诊治15例APL患儿。单剂AS2O3诱导分化治疗,达到完全缓解后采用DA/HA/IDA方案与ATRA/AS2O3序贯治疗。辅以小分子肝素抗凝治疗,必要时加用白细胞置换术。观察其临床特点、血常规、出凝血指标、肝肾功能、心电图,监测PML/RARa融合基因、染色体的变化。结果15例患儿2例早期死亡,12例PML/RARa融合基因阳性者均得到完全缓解。AS2O3治疗1周白细胞上升最高,治疗两周开始下降,D-Dimer正常,出血倾向减轻或消失。治疗6~8周CR。结论单剂AS2O3诱导分化治疗,对PML/RARa融合基因阳性,存在染色体t(15;17)患者,均能完全缓解率。且毒副反应较小。早期小分子肝素钙应用配合血浆输注,必要时行白细胞单采术帮助大多数患儿安全度过DIC,获得长期生存。
Objective To investigate the clinical features and treatment of childhood acute promyelocytic leukemia (APL). Methods From June 2002 to March 2007, 15 children with APL were diagnosed and treated in our hospital. A single dose of AS2O3 induced differentiation treatment, to achieve complete remission using DA / HA / IDA program and ATRA / AS2O3 sequential treatment. Supplemented by small molecular heparin anticoagulant therapy, if necessary, add leukocyte replacement surgery. Observed its clinical features, blood, coagulation index, liver and kidney function, electrocardiogram, monitoring PML / RARa fusion gene, chromosome changes. Results Two of 15 children died early and all 12 patients with positive PML / RARa fusion gene were completely relieved. One week after AS2O3 treatment, the white blood cells increased the most, the treatment began to decline in two weeks, D-Dimer was normal, and the bleeding tended to reduce or disappear. Treatment of 6 to 8 weeks CR. Conclusions The single-dose AS2O3 induced differentiation therapy can achieve a complete remission rate in patients with positive PML / RARa fusion gene and chromosome t (15; 17). And less toxic side effects. Early application of small molecule heparin with plasma infusion, if necessary, leukoplakia surgery to help most children safely through DIC, access to long-term survival.