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No prognostic effect of additional chromosomal abnormalities in children with acute lymphoblastic leukemia and 11q23 abnormalities

机译:儿童急性淋巴细胞白血病和11q23异常患儿的其他染色体异常无预后影响

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This study characterized the additional chromosomal abnormalities (ACA) associated with 11q23 rearrangements in 450 infants and children with acute lymphoblastic leukemia (ALL) and examined the impact of these ACA on survival. Overall, 213 (47%) cases had ACA but the incidence varied according to patient age and 11q23 subgroup. Infants and patients with t(4;11)(q21;q23) had the lowest incidence of ACA (50/182 (27%) and 57/216 (26%) respectively), whereas patients with del(11)(q23) had the highest incidence (66/93 (71%)). Del(11)(q23) abnormalities were heterogeneous and occasionally secondary to t(9;22)(q34;q11.2). Thus, patients with del(11)(q23) comprised a separate biological entity, which was clearly distinct from those with an 11q23 translocation. The most frequent specific ACA were trisomy X (n=38), abnormal 12p (n=32), abnormal 9p (n=28) and del(6q) (n=19). The presence of ACA did not change the 5 year event-free survival estimates among children (56% (95% CI 46–65%) vs 62% (54–69%)) or infants (22% (15–29%) vs 18% (9–29%)), nor when the different 11q23 subgroups were analyzed separately. This study has conclusively demonstrated that there is no prognostic effect of secondary chromosomal changes in association with 11q23 abnormalities in childhood ALL. However, characterization of these ACA is important to determine their potential role in initiation of MLL driven leukemogenesis.
机译:这项研究对450例急性淋巴细胞白血病(ALL)婴幼儿的11q23重排相关的额外染色体异常(ACA)进行了研究,并研究了这些ACA对生存的影响。总体上,有213例(47%)患有ACA,但其发病率因患者年龄和11q23亚组而异。婴儿和t(4; 11)(q21; q23)患者的ACA发生率最低(分别为50/182(27%)和57/216(26%)),而del(11)( q23)的发生率最高(66/93(71%))。 Del(11)(q23)异常是异类的,偶发于t(9; 22)(q34; q11.2)。因此,患有del(11)(q23)的患者包含一个单独的生物学实体,这明显不同于具有11q23易位的患者。最常见的特定ACA是X三体性(n = 38),异常12p(n = 32),异常9p(n = 28)和del(6q)(n = 19)。 ACA的存在并未改变儿童(56%(95%CI 46–65%)相对于62%(54-69%))或婴儿(22%)的5年无事件生存率(15–29%)vs 18%(9–29%)),也没有分别分析不同的11q23子组的情况。这项研究结论性地表明,继发性染色体改变与儿童ALL的11q23异常没有预后影响。但是,这些ACA的表征对于确定其在MLL驱动的白血病发生的起始中的潜在作用很重要。

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