Outcome of treatment in children with philadelphia chromosome-positive acute lymphoblastic leukemia
Outcome of treatment in children with philadelphia chromosome-positive acute lymphoblastic leukemia
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DOI：
10.1056/nejm200004063421402
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发表时间：
2000-04-06
影响因子：
158.5
通讯作者：
Masera, G
中科院分区：
文献类型：
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作者：
Aricò, M;Valsecchi, MG;Masera, G
背景：费城染色体阳性急性淋巴细胞白血病（Ph 阳性 ALL）儿童预后较差，对于这种 ALL 变型的最佳治疗方案尚未达成共识。方法：我们回顾了 10 个研究组或大型单一机构 1986 年至 1996 年间接受强化化疗（伴或不伴骨髓移植）的 Ph 阳性 ALL 患者的病历。数据涉及 326 名儿童和年轻人，年龄范围为根据标准预后因素和治疗类型，对 0.4 至 19.9 年（中位数为 8.1）进行分析，以确定完全缓解率以及无事件、无病和总生存的概率。 结果：诱导化疗后完全缓解的 267 名患者（82%）根据诊断时的年龄和白细胞计数分为三个亚组：预后最好的患者（白细胞计数低于50,000/立方毫米且年龄低于10岁的患者）；具有中等预后的患者（中等风险特征；92 名患者）；以及预后最差的患者（白细胞计数超过每立方毫米 100,000 个；80 名患者）。五年无病生存率 (+/-SE) 估计为 49+/-5%（对于预后最好的患者）、30+/-5%（对于预后中等的患者）和 20+/-5%（对于预后最差的患者）（P
Background: Children with Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph-positive ALL) have a poor prognosis, and there is no consensus on the optimal treatment for this variant of ALL.Methods: We reviewed the medical records of patients with Ph-positive ALL who were treated with intensive chemotherapy, with or without bone marrow transplantation, by 10 study groups or large single institutions from 1986 to 1996. Data on 326 children and young adults, who ranged in age from 0.4 to 19.9 years (median, 8.1), were analyzed to determine the rate of complete remission and the probability of event-free, disease-free, and overall survival according to standard prognostic factors and type of treatment.Results: The 267 patients who had a complete remission after induction chemotherapy (82 percent) were stratified into three subgroups according to the age and leukocyte count at the time of diagnosis: those with the best prognosis (a leukocyte count of less than 50,000 per cubic millimeter and an age of less than 10 years; 95 patients); those with an intermediate prognosis (intermediate-risk features; 92 patients); and those with the worst prognosis (a leukocyte count of more than 100,000 per cubic millimeter; 80 patients). The estimates of disease-free survival at five years (+/-SE) were 49+/-5 percent (for patients with the best prognosis), 30+/-5 percent (for those with an intermediate prognosis), and 20+/-5 percent (for those with the worst prognosis) (P