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Prognostic relevance of Wilms tumor 1 (WT1) gene Exon 7 mutations in-patient with cytogenetically normal acute myeloid leukemia

机译:细胞遗传学上正常的急性髓细胞性白血病患者中Wilms肿瘤1(WT1)基因外显子7突变的预后相关性

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摘要

This study aimed to assess the prognostic influences of Wilms tumor 1 (WT1) gene mutations in cytogenetically normal acute myeloid leukemia (CN-AML) among Egyptian patients. Exon 7 of WT1 was screened for mutations in samples from 82 CN-AML patients out of 203 newly diagnosed AML patients, using a high-resolution capillary electrophoresis. Seven out of 82 AML patients (8.3 %) harbored WT1 mutations. There was no significant difference between the mutant WT1 and wild type AML patients as regard age, sex, French–American–British subtypes and the prevalence of success of induction remission therapy (P < 0.5). AML patients with mutant WT1 had shorter overall survival as compared to those patients with wild WT1 (HR = 1.38; 95 % CI 4.79–6.86; P = 0.004). In conclusion, CN-AML patients with WT1 gene mutation have poor clinical outcome. We recommend testing the WT1 mutations as part of molecularly based risk assessment and risk-adapted treatment stratification of patients with CN-AML.
机译:这项研究旨在评估Wilms肿瘤1(WT1)基因突变在埃及患者中的细胞遗传学上正常的急性髓细胞性白血病(CN-AML)中的预后影响。使用高分辨率毛细管电泳,从203名新诊断的AML患者中筛选了WT1的第7外显子中82位CN-AML患者的样本中的突变。 82名AML患者中有7名(8.3%)携带WT1突变。 WT1突变型和野生型AML患者之间在年龄,性别,法国-美国-英国亚型和诱导缓解治疗成功率方面无显着差异(P <0.5)。与野生型WT1的患者相比,突变型WT1的AML患者的总生存期更短(HR = 1.38; 95%CI 4.79–6.86; P = 0.004)。总之,具有WT1基因突变的CN-AML患者临床预后较差。我们建议测试WT1突变,作为基于分子的风险评估和风险适应性CN-AML患者治疗分层的一部分。

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