Long-term results of an ultra low-dose cytarabine-based regimen for the treatment of acute megakaryoblastic leukaemia in children with Down syndrome

Long-term results of an ultra low-dose cytarabine-based regimen for the treatment of acute megakaryoblastic leukaemia in children with Down syndrome
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DOI:
10.1111/j.1365-2141.2006.06097.x
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发表时间:
2006-06-01
影响因子:
6.5
通讯作者:
Hitzler, Johann
Hitzler, Johann
中科院分区:
医学2区
文献类型:
--
作者:
Al-Ahmari, Ali;Shah, Niketa;Hitzler, Johann

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患有急性巨核母细胞白血病(AMKL)和唐氏综合征(DS)的儿童对化疗反应良好,可能是由于白血病原细胞对阿糖胞苷的敏感性增加。相比之下,剂量强化方法导致该组中不成比例的治疗相关死亡率。采用低剂量化疗方案,包括阿糖胞苷(10mg /m(2)/剂量)、视黄醇棕榈酸酯和长春新碱或标准化疗,回顾性比较AMKL和DS患儿的生存率。两组5年无事件(67 +/- 11%)和总生存率(77 +/- 10%)无显著差异。因此,进一步降低退行性痴呆患儿AMKL的治疗强度似乎是可行的。
Children with acute megakaryoblastic leukaemia (AMKL) and Down syndrome (DS) show a favourable response to chemotherapy, probably due to increased sensitivity of the leukaemic blasts to cytarabine. In contrast, dose-intensive approaches have resulted in disproportionate treatment-related mortality in this group. The survival of children with AMKL and DS was retrospectively compared following treatment with a low-dose chemotherapy protocol, consisting of cytarabine (10 mg/m(2)/dose), retinylpalmitate and vincristine or standard chemotherapy. Event-free (67 +/- 11%) and overall survival (77 +/- 10%) at 5 years were not significantly different in both groups. Further reduction of treatment intensity in AMKL of children with DS, therefore, appears feasible.