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儿童急性淋巴细胞白血病(ALL)的治疗:意大利十年经验总结。意大利儿科血液肿瘤协会(AIEOP)ALL指导委员会

Treating childhood acute lymphoblastic leukaemia (ALL): summary of ten years' experience in Italy. ALL Steering Committee of the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP).

作者信息

Paolucci G, Masera G, Vecchi V, Marsoni S, Pession A, Zurlo M G

机构信息

Clinica Pediatrica, Ospedale S. Orsola, Bologna, Italy.

出版信息

Med Pediatr Oncol. 1989;17(2):83-91. doi: 10.1002/mpo.2950170203.

DOI:10.1002/mpo.2950170203
PMID:2704341
Abstract

Between 1976 and 1986, 2,093 children with ALL were enrolled in three consecutive generations of trials conducted by the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP). A 50% event-free survival at 5 years was achieved overall in this population, approximately accounting for more than 50% of the entire childhood ALL population in Italy. Participation in the group protocols increased from the original seven founding centers to the current 37 institutions. Results in the standard population (non-T immunophenotype, non-FAB L3, and less than 50,000 white blood cells (WBC/ml) were considerably better with more recent, more aggressive protocols. The two major results in this population (N = 540) were a relatively low incidence (8% at 5 years) of central nervous system (CNS) relapse in the "good"-risk population (less than 10,000 WBC, ages 3-6 years, and FAB L1), without the use of cranial irradiation, and a projected 4-year disease-free interval for bone-marrow relapse of 80% in the "average"-risk group, where a three-drug reinduction program was adopted after consolidation. Overall, the event-free survival of the most recent generation (protocol 82, median follow-up time of 38 months) is 66% at 4 years (95% confidence limits [CL] 61-71). Based on these 10 years of experience, the general strategy of the group for the 90s is outlined and discussed.

摘要

1976年至1986年期间,2093名急性淋巴细胞白血病患儿参加了由意大利儿童血液肿瘤协会(AIEOP)开展的连续三代试验。该人群总体上实现了5年无事件生存率为50%,约占意大利整个儿童急性淋巴细胞白血病患者总数的50%以上。参与组内方案研究的机构从最初的7个创始中心增加到了目前的37个。采用更新的、更积极的方案后,标准人群(非T免疫表型、非FAB L3以及白细胞计数低于50,000个/毫升)的治疗结果有了显著改善。该人群(N = 540)的两个主要成果是,在“低危”人群(白细胞计数低于10,000个、年龄3至6岁、FAB L1)中,未采用颅脑照射时中枢神经系统(CNS)复发率相对较低(5年时为8%);在“中危”组中,采用巩固治疗后三药再诱导方案,预计骨髓复发的4年无病间期为80%。总体而言,最新一代方案(方案82,中位随访时间38个月)4年时的无事件生存率为66%(95%置信区间[CL] 61 - 71)。基于这10年的经验,概述并讨论了该组织90年代的总体策略。

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