Prognostic factors in childhood acute lymphoblastic leukemia.

Hematologic pathology Pub Date : 1993-01-01
R C Ribeiro, C H Pui
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Abstract

Treatment efficacy alters the impact of most prognostic factors. Among clinical features, only age and leukocyte count remain prognostically important. Immunophenotyping is useful for ALL classification and for assignment to specific therapy regimens, but, with the possible exception of CD10 expression, has little prognostic importance in the context of contemporary phenotype- and risk-directed therapy. Cytogenetic features are useful for risk assignment. Hyperdiploidy > 50 chromosomes is associated with a favorable prognosis, whereas Ph+ chromosome and t(4;11) confer an adverse prognosis. Pre-B cases with the t(1;19) do not fare well with antimetabolite-based therapy and should be treated with additional classes of chemotherapeutic agents. Finally, certain specific rearrangements such as dic(9;12) may in fact be associated with favorable prognosis. With the exception of treatment for B-cell ALL (and perhaps transitional pre-B ALL), phenotype- or genotype-directed therapies have not been successfully devised. Selection of treatment for individual patients, therefore, should be based on their estimated risk of failure. For patients with very high-risk leukemia (i.e., those with > 70% likelihood of treatment failure), the use of experimental therapeutic strategies, despite the potential for acute and long-term disabilities, may be justified. For the subset of children in the lower-risk category (< 20% probability of failure), antimetabolite-based therapy should be employed to minimize long-term sequelae.

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儿童急性淋巴细胞白血病的预后因素。
治疗效果改变了大多数预后因素的影响。在临床特征中,只有年龄和白细胞计数对预后有重要影响。免疫表型分型对ALL的分类和特定治疗方案的分配是有用的,但是,除了CD10表达的可能例外,在当代表型和风险导向治疗的背景下,免疫表型分型对预后的重要性很小。细胞遗传学特征对风险分配是有用的。> 50条染色体的高二倍体与预后良好相关,而Ph+染色体和t(4;11)则导致预后不良。b前t(1;19)患者使用基于抗代谢物的治疗效果不佳,应使用其他类型的化疗药物进行治疗。最后,某些特定的重排,如dic(9;12)实际上可能与良好的预后有关。除了b细胞ALL(可能还有过渡性b细胞前ALL)的治疗外,表型或基因型导向的治疗尚未成功设计。因此,对个别患者的治疗选择应基于他们估计的失败风险。对于高危白血病患者(即治疗失败可能性大于70%的患者),尽管可能导致急性和长期残疾,但使用实验性治疗策略可能是合理的。对于低风险类别的儿童亚群(失败概率< 20%),应采用基于抗代谢物的治疗,以尽量减少长期后遗症。
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