Prevalence and Prognostic Impact of Wilms' Tumor 1 (Wt1) Gene, Including Snp Rs16754 in Cytogenetically Normal Acute Myeloblastic Leukemia (Cn-Aml): An Iranian Experience Publisher Pubmed



Toogeh G^{1, 2} ; Ramzi M³ ; Faranoush M¹ ; Amirizadeh N¹ ; Haghpanah S³ ; Moghadam M³ ; Cohan N¹ Show Affiliations
Source: Clinical Lymphoma# Myeloma and Leukemia Published:2016

Abstract

Background The aim of this study was to evaluate the effect of Wilms' tumor 1 (WT1) gene mutations in adult cytogenetically normal acute myeloblastic leukemia (CN-AML) patients on survival and clinical outcome. Patients and Methods A total of 88 untreated Iranian adult patients with CN-AML were selected as a study group. Exons 7 (including the SNP rs16754), 8, and 9 as a WT1 gene hotspot region were evaluated by polymerase chain reaction and direct sequencing for detection of mutations. Response to treatment and clinical outcome including overall survival (OS) and disease-free survival (DFS) were evaluated according to WT1 gene mutational status. Results WT1 gene mutations were found in 12.5% of patients, most of which were found in exon 7. Complete remission was lower and relapse was higher in patients with WT1 gene mutation compared with WT1 gene wild type patients. OS and DFS was significantly lower in patients with WT1 gene mutation compared with patients with WT1 gene wild type (P <.001). Also, we did not find any significant effects of SNP rs16754 in exon 7 on clinical outcome and survival in patients with CN-AML. Conclusion WT1 gene mutations are a predictor indicator of a poor prognosis factor in CN-AML patients. It is recommended that WT1 gene mutations be included in the molecular testing panel in order to better diagnose and confirm their prognostic significance for better management and treatment strategy. © 2016 Elsevier Inc. All rights reserved.