Al-Nasser Abdallah, El-Solh Hassan, De Vol Edward, El-Hassan Ibrahim, Alzahrani Ali, Al-Sudairy Reem, Al-Mahr Mohammed, Al-Musa Abdulrahman, Al-Jefri Abdulla, Saleh Mahasen, Rifai Samira, Belgaumi Asim, Osman Layla, Ashraf Khairy, Salim Mohammed, Silo Ameurfina, Roberts George
Department of Pediatric Hematology/Oncology, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia.
Ann Saudi Med. 2008 Jul-Aug;28(4):251-9. doi: 10.5144/0256-4947.2008.251.
Because of the need for more comprehensive information on the least toxic and most effective forms of therapy for children with acute lymphoblastic leukemia (ALL), we reviewed our experience in the treatment of children with ALL at King Faisal Specialist Hospital and Research Centre (KFSH&RC) and King Fahad National Center for Children's Cancer and Research (KFNCCC&R) over a period of 18 years with a focus on patient characteristics and outcome.
During the period of 1981 to 1998, records of children with ALL were retrospectively reviewed with respect to clinical presentation, laboratory findings, risk factors, stratification, therapy and outcome. The protocols used in treatment included 4 local protocols (KFSH 81, 84, 87 and 90), and subsequently, Children's Cancer Group (CCG) protocols, and these were grouped as Era 1 (1981-1992) and Era 2 (1993-1998).
Of 509 children with ALL treated during this period, 316 were treated using local protocols and 193 using CCG protocols. Drugs used in Era 1 included a 4-drug induction using etoposid (VP-16) instead of L-asparaginase. Consolidation was based on high dose methotrexate (MTX) 1 g/m(2) and maintenance was based on oral mercaptopurine (6-MP) and MTX with periodic pulses using intravenous teniposide (VM-26), Ara-C, L-asparaginase, adriamycin, prednisone, VP-16 and cyclophosphamide. International protocols were introduced in Era 2, which was also marked by intensification of early treatment, a wider selection of cytoreductive agents, and the alternating use of non-cross-resistant pairs of drugs during the post-remission period. The end-of-induction remission rate improved from 90% in Era 1 to 95% in Era 2, which was of borderline statistical significance (P=.049). The 5-year event-free survival (EFS) improved from 30.6% in Era 1 to 64.2% in Era 2 (P<.001). Improvement in outcome was achieved without any significant increase in morbidity or mortality, due to improvement in both systemic therapy and supportive care. The most important independent prognostic factors were intensity of therapy, poor risk category assignment and CNS disease at diagnosis.
Outcome in children with ALL has improved because of intensification of treatment protocols and better supportive care.
由于需要获取更多关于急性淋巴细胞白血病（ALL）患儿毒性最小且最有效的治疗形式的全面信息，我们回顾了在费萨尔国王专科医院及研究中心（KFSH&RC）和法赫德国王儿童癌症与研究国家中心（KFNCCC&R）对ALL患儿18年的治疗经验，重点关注患者特征和治疗结果。
在1981年至1998年期间，对ALL患儿的记录进行回顾性分析，内容包括临床表现、实验室检查结果、危险因素、分层、治疗及结果。治疗中使用的方案包括4个本地方案（KFSH 81、84、87和90），随后采用儿童癌症组（CCG）方案，这些方案被分为第1阶段（1981 - 1992年）和第2阶段（1993 - 1998年）。
在此期间治疗的509例ALL患儿中，316例采用本地方案治疗，193例采用CCG方案治疗。第1阶段使用的药物包括用依托泊苷（VP - 16）替代L - 天冬酰胺酶进行4药诱导。巩固治疗基于高剂量甲氨蝶呤（MTX）1 g/m²，维持治疗基于口服巯嘌呤（6 - MP）和MTX，并定期静脉注射替尼泊苷（VM - 26）、阿糖胞苷、L - 天冬酰胺酶、阿霉素、泼尼松、VP - 16和环磷酰胺。第2阶段引入了国际方案，其特点还包括强化早期治疗、更广泛地选择细胞减灭剂以及在缓解后期交替使用非交叉耐药的药物组合。诱导结束时的缓解率从第1阶段的90%提高到第2阶段的95%，具有临界统计学意义（P = 0.049）。5年无事件生存率（EFS）从第1阶段的30.6%提高到第2阶段的64.2%（P < 0.001）。由于全身治疗和支持治疗的改善，在发病率和死亡率没有显著增加的情况下实现了治疗结果的改善。最重要的独立预后因素是治疗强度、不良风险类别划分和诊断时的中枢神经系统疾病。
由于治疗方案的强化和更好的支持治疗，ALL患儿的治疗结果得到了改善。
