期刊
CANCER
卷 115, 期 16, 页码 3719-3727出版社
WILEY
DOI: 10.1002/cncr.24442
关键词
acute myeloid leukemia; adult; Wilms tumor 1 mutations; frequency; prognosis
类别
资金
- Fondation de France (Leukemia Committee)
- North-West Canecropole (Onco-Hematology Axis)
BACKGROUND: Wilms tumor 1 (WT1) is a transcription factor that is overexpressed in most acute myeloid leukemias (AMLs). Recently, 2 groups reported that WT1 mutations occur in approximately 10% of normal karyotype AMLs and are an independent predictor of poor outcome in this subgroup of patients with AML. METHODS: The authors studied a cohort of 268 young adults (ages 15-50 years) with AML who were treated on the Acute Leukemia French Association 9802 trial. WT1 exon 7 and 9 mutations were screened retrospectively by polymerase chain reaction and direct sequencing. The patients also were assessed for the presence of the fms-related tyrosine kinase 3 internal tandem duplication (FLT3-ITD), FLT3-D835/1836, nucleophosmin 1 (NPM7), and CCAAT/enhancer binding protein alpha (CEBPA) mutations. RESULTS: WT1 mutations were identified in 14 patients (5%) and were associated with a younger age (P = .02) and an FLT3-ITD (P = .03). No mutation was detected in patients who had favorable cytogenetics. Patients who had WT1 mutations had a shorter overall survival at 4 years (22% vs 56%; P = .01) and a higher risk of recurrence at 4 years (82% vs 46%; P = .0008) compared with patients who had wild-type WT1. Within the subgroup of patients who had normal karyotype AML (n = 106), WTI mutation was identified as an independent adverse prognostic factor for the risk of recurrence. CONCLUSIONS: The current results indicted that WTI mutations represent an adverse prognostic factor in young adults with AML. Prospective trials should confirm the clinical relevance of WT1 mutations in relation to other prognostic factors in patients with AML. Cancer 2009;115:3719-27. (C) 2009 American Cancer Society.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据