15例儿童急性早幼粒细胞性白血病临床及治疗研究

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。

Study on the clinical characteristics and treatments of acute Promyelocytic Leakemia in 15 children

Objective To evaluate the clinical characters and treatments in acute Promyelocytic Leakemia of child. Methods 15 APL patients were selected from June, 2002 to March, 2007. AS2O3 was used for induction. DA/HA/IDA and ARTA/AS2O3 were administered alternately after complete remission by AS2O3. Nadroparin Calcium was treated in antagonizing coagulative blood. Replacement of white cell was used necessary. Clinical character and blood count and bone marrow smear and coagulation and fibrinolysis and fuction of hepatic and nephric and electorocardiograph were observed. The change about RT-PCR of PML/RAR a and chromogome were monitored. Results two patients died after the treatment due to intracranial hemorrhage, 12 patiens with PML/RAR a fuction gene being positive achieved CR. Hyperleukocytosis emerged in one week after treatment of AS2O3. After two weeks, it began to descend and D-Dimer was normal. Hemorrhage alleriated or disappeared. CR were achieved after 6 - 8 weeks. Conclusion 12 patiens with with PML/RAR a fuction gene being positive were all CR during Induction with AS2O3. The side effect was less than the combined chemotherapy. The use of Nadroparin Calcium and plasma and replacement of white cell could help more children pass DIC safest . Long-term subsist was achieved.