糖皮质激素反应性与急性淋巴细胞白血病患儿预后的关系

目的评价糖皮质激素反应性与急性淋巴细胞白血病患儿持续缓解、复发、死亡等预后的关系。方法收集中山大学附属第一医院2008年6月至2011年12月146例住院急性淋巴细胞白血病患儿,通过泼尼松诱导试验初步判断激素的反应性,根据激素反应性不同分组并结合相应的实验室指标:初始外周血白细胞数,初始外周血幼稚淋巴细胞数,外周血幼稚淋巴细胞百分比,融合基因MLL及BCR/ABL,化疗后第8、26天骨髓象,从而评价急性淋巴细胞白血病患儿糖皮质激素反应性与预后的关系。结果 146例患儿中,糖皮质激素反应性敏感(PR+)者为121例(82.88%),其中107例(88.43%)处于持续缓解状态,复发11例,死亡2例,放弃治疗1例。糖皮质激素反应性不敏感(PR-)者为25例(17.12%),其中持续缓解13例,复发6例,死亡5例,放弃治疗1例。PR+组复发死亡率明显低于PR-组,其中两组患儿的初始外周血白细胞数、初始外周血幼稚淋巴细胞数、外周血幼稚淋巴细胞百分比、化疗后第8、26天骨髓象、危险度的分级差异有统计学意义,且复发组的初始幼稚淋巴细胞百分比明显高于未复发组。结论通过泼尼松诱导试验结合一系列实验室指标可判断糖皮质激素的反应性,从而指导疾病的治疗和预测疾病转归。

Relationship between glucocorticoid response and the prognosis of childhood acute lymphoblastic leukemia

Objective To assess the value of glucocorticoid response in children with acute lymphoblastic leu- kemia（ALL）. Methods From June 2008 to December 2011, children with newly diagnosed ALL were divided into two groups according to prednisone induction test and the corresponding laboratory characteristics such as： initial white blood celI（WBC） count, initial peripheral blast count, the percentage of initial peripheral blast, MLL gene and BCR/ABL gene, bone marrow characteristics at the 8th and 26th day after chemotherapy, to further evaluate the glucocorticoid response. Results Of 146 children, 121（82. 880//oo） cases were classified as prednisone good responder （PRnu） , including 107 cases of continuous complete remission（CCR）, 1 case gave up therapy, 11 cases in relapse and 2 cases died. While 25（17.12~） cases were prednisone poor responders（PR--）, including 13 cases of CCR, 6 cases in relapse, 5 cases died and 1 case gave up therapy. Initial WBC count, initial peripheral blast count, the per- centage of initial peripheral blast, bone marrow examined on the 8th and 26th day after treatment and risk stratifica- tion were significantly different between PR＋ and PR-- group. And the patients in relapse had higher initial blast count than children in CCR. Conclusion Response to prednisone, identified by prednisone induction test and other laboratory tests, could be used as a stratification factor in risk-adapted treatment protocols and good prognostic fac- tor in childhood ALL.