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首页> 外文期刊>Acta paediatrica. Supplement >Infant leukaemia: Clinical, biological and therapeutic advances
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Infant leukaemia: Clinical, biological and therapeutic advances

机译:婴儿白血病:临床,生物学和治疗学进展

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Infant acute lymphoid leukaemia (IALL) represents a distinct subset with an extremely poor response to therapy, despite major progress in the treatment of childhood leukaemia. However, several studies have shown that, even in this generally considered homogeneous group, a distinction could be made with regard to prognosis. The outcome of IALL patients with ALL-1IMLL rearrangements at the Ilq23 cytogenetic band, early pre-B immunophenotype, high WBC count and age below 6 mo is significantly worse than in patients without these characteristics, and current therapies appear inadequate in a significant number of cases. Therefore, an international protocol (Interfant 99) was recently started, using a more aggressive approach, which included lymphoid- and myeloid-specific drugs, and indications for stem-cell transplantation, We reviewed the clinical characteristics of the disease, the results of several recent international clinical trials, and our experience with 16 infants with acute lymphoid leukaemia diagnosed and treated at our institution.Conclusion: It is extremely important to stratify patients for prognosis, taking into account clinical and biological variables with independent prognostic value. The aim is to select more adequate, risk-adapted, therapeutic strategies which also consider related or unrelated bone marrow transplant consolidation for patients with very poor prognosis.
机译:尽管婴儿期白血病的治疗取得了重大进展,但婴儿急性淋巴白血病(IALL)代表了对治疗反应极差的独特亚型。但是,一些研究表明,即使在这个通常被认为是同质的人群中,也可以在预后方面做出区分。 IALL患者在Ilq23细胞遗传带处发生ALL-1IMLL重排,B前早期免疫表型,WBC计数高且年龄低于6 mo的IALL患者的结果显着低于没有这些特征的患者,目前的治疗方法在很多患者中均不足案件。因此,最近开始了一项国际协议(Interfant 99),采用了更具侵略性的方法,其中包括淋巴样和髓样特异性药物以及干细胞移植的适应症。我们回顾了该疾病的临床特征,总结了几种方法的结果。最近的国际临床试验,以及我们在本机构诊断和治疗的16例急性淋巴白血病婴儿的经验。结论:考虑到具有独立预后价值的临床和生物学变量,对患者的预后进行分层非常重要。目的是为预后极差的患者选择更适当的,适应风险的治疗策略,其中还应考虑相关或不相关的骨髓移植巩固。

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