Cortes J E, Kantarjian H M
Department of Hematology, University of Texas M.D. Anderson Cancer Center, Houston 77030, USA.
Cancer. 1995 Dec 15;76(12):2393-417. doi: 10.1002/1097-0142(19951215)76:12<2393::aid-cncr2820761203>3.0.co;2-p.
Acute lymphoblastic leukemia (ALL) is the most common malignancy in children. It is now curable in 60-70% of children. Most of the current understanding of the biology and treatment of ALL originates from studies of children. In adults, although much progress has been achieved, ALL is curable in only 20-35% of patients.
A review of the biology and treatment of ALL from the English literature was performed.
Immunophenotypic and cytogenetic analyses of ALL have contributed to a more rational classification of ALL. These analyses have identified subgroups with poor prognosis or with different therapeutic requirements. Overall, 60-70% of adults with ALL have poor prognostic features, including older age, a high leukocyte count, non-T-cell immunophenotype, Ph-positive genotype, and longer time to achieve a complete remission. These patients have a cure rate of 20-25%, whereas those without these risk factors, have a 60-70% probability of survival. The use of more intensive induction regimens with growth factor support may improve survival rates. Also, intensive consolidation-intensification may improve survival rates. Most patients benefit from maintenance therapy, but the dose schedule must be optimized. Central nervous system (CNS) prophylaxis is beneficial, particularly for patients with a high risk for CNS relapse and when introduced early during induction of remission. Patients with high risk characteristics may benefit from allogeneic bone marrow transplantation (BMT) during first remission, and all other patients may benefit from it during first or subsequent relapse. Autologous BMT may be a valuable option for poor compliant patients.
Although the prognosis of patients with ALL has improved markedly during the past decades, newer strategies, including more dose-intensive therapy, the search for new drugs, and more target-specific therapy, are needed to improve the current cure rates.
急性淋巴细胞白血病（ALL）是儿童中最常见的恶性肿瘤。目前，60%至70%的儿童患者可治愈。目前对ALL生物学特性和治疗方法的大部分认识都源于对儿童的研究。在成人中，尽管已取得很大进展，但ALL患者的治愈率仅为20%至35%。
对英文文献中ALL的生物学特性和治疗方法进行综述。
ALL的免疫表型和细胞遗传学分析有助于对ALL进行更合理的分类。这些分析确定了预后不良或有不同治疗需求的亚组。总体而言，60%至70%的成人ALL患者具有不良预后特征，包括年龄较大、白细胞计数高、非T细胞免疫表型、Ph阳性基因型以及达到完全缓解所需时间较长。这些患者的治愈率为20%至25%，而没有这些危险因素的患者存活概率为60%至70%。使用更强化的诱导方案并给予生长因子支持可能会提高生存率。此外，强化巩固-强化治疗也可能提高生存率。大多数患者从维持治疗中获益，但剂量方案必须优化。中枢神经系统（CNS）预防是有益的，特别是对于CNS复发风险高的患者以及在诱导缓解早期进行预防时。具有高风险特征的患者在首次缓解期可能从异基因骨髓移植（BMT）中获益，而所有其他患者在首次或后续复发时可能从中获益。自体BMT对于依从性差的患者可能是一个有价值的选择。
尽管在过去几十年中ALL患者的预后有了显著改善，但仍需要更新的策略，包括更强剂量的治疗、寻找新药以及更具靶向性的治疗，以提高当前的治愈率。
