Prognosis of children with acute lymphoblastic leukemia (ALL) and intrachromosomal amplification of chromosome 21 (iAMP21)
Prognosis of children with acute lymphoblastic leukemia (ALL) and intrachromosomal amplification of chromosome 21 (iAMP21)
复制标题
DOI：
10.1182/blood-2006-08-040436
复制
发表时间：
2007-03-15
期刊：
影响因子：
20.3
通讯作者：
Harrison, Christine J.
中科院分区：
文献类型：
--
作者：
Moorman, Anthony V.;Richards, Susan M.;Harrison, Christine J.
急性淋巴细胞白血病（ALL）和21号染色体内扩增（iAMP 21）的患者构成了一个新的和独特的生物学亚群。我们前瞻性筛选了1630例（84%）接受英国MRC ALL 97方案治疗的iAMP 21患者，并在此提供了28例（2%）发现存在这种异常的儿童的人口统计学、临床和生存数据。与无此异常的儿童相比，他们有一个共同的或前BALL免疫表型，年龄明显更大（中位数9岁比5岁），白色细胞计数更低（中位数3.9比12.4）。值得注意的是，iAMP 21患者的5年无事件生存率和总生存率显著低于其他患者：分别为29%（95%置信区间[CI]，13%-48%）vs 78%（95% CI，76%-80%）和71%（95% CI，51%-84%）vs 87%（95% CI，85%-88%）。由于复发风险增加了3倍，目前英国MRC ALL 2003试验招募的新诊断iAMP 21患者正在高风险组接受治疗，并考虑在首次缓解时进行骨髓移植。
Patients with acute lymphoblastic leukemia (ALL) and an intrachromosomal amplification of chromosome 21 (iAMP21) comprise a novel and distinct biological subgroup. We prospectively screened 1630 (84%) patients treated on the UK MRC ALL97 protocol for iAMP21 and herein present demographic, clinical, and survival data on the 28 (2%) children found to harbor this abnormality. They had a common or pre-BALL immunophenotype, were significantly older (median 9 years vs 5 years), and had a lower white cell count (median 3.9 vs 12.4) compared with children without this abnormality. Notably, patients with iAMP21 had a significantly inferior event-free and overall survival at 5 years compared with other patients: 29% (95% confidence interval [CI], 13%-48%) versus 78% (95% Cl, 76%-80%) and 71% (95% Cl, 51%-84%) versus 87% (95% Cl, 85%-88%), respectively. As a result of this 3-fold increase in relapse risk, newly diagnosed patients with iAMP21 recruited to the current UK MRC ALL2003 trial are being treated on the high-risk arm and are considered for bone marrow transplantation in first remission.