title: Incidence and prognostic importance of molecular genetic defects in children with acute myeloblastic leukemia.
abstract: BACKGROUND
  Acute myeloblastic leukemia (AML) accounts for 15 to 25 percent of childhood acute leukemias. The most common genetic abnormalities seen in pediatric AML patients are AML1-ETO, PML-RARα and CBFB-MYH11 genes resulting in t(8;21), t(15;17) and inv(16). These genetic defects are seen in approximately 20-25% of AML patients.
  
  
  OBJECTIVE
  We investigated in this study, incidence and prognostic significance of the AML1-ETO, PML-RARα and CBFB-MYH11 genes in children with AML.
  
  
  METHODS
  The authors analyzed 34 children with AML using the real time-polymerase chain reaction for AML1-ETO, PML-RARα and CBFB-MYH11 genes.
  
  
  RESULTS
  Of the patients, 8.8% were positive for t(8;21), 8.8% for t(15;17) and 3% for inv(16). There were a statistically significant differences between 48 month overall survival rates of the patients positive and negative for t(8;21), t(15;17) and inv(16).
  
  
  CONCLUSIONS
  It was concluded that t(15;17), t(8;21) and inv(16) impact on disease prognosis positively, but comprehensive studies with larger patient series are now needed for confirmation.
authors.affiliation: Department of Pediatric Oncology and Pediatric Bone Marrow Transplantaion Unit, Faculty of Medicine, Cukurova University, Adana, Turkey.
authors.forename: Mustafa
authors.initials: M
authors.lastname: Kömür
authors.forename: Fatih
authors.initials: F
authors.lastname: Erbey
authors.forename: Ibrahim
authors.initials: I
authors.lastname: Bayram
authors.forename: Atila
authors.initials: A
authors.lastname: Tanyeli
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002884:Chromosomes, Human, Pair 15; D002898:Chromosomes, Human, Pair 8; D050676:Core Binding Factor Alpha 2 Subunit / Q000235:genetics; D005260:Female; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D015470:Leukemia, Myeloid, Acute / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics*; D008297:Male; D009154:Mutation / Q000235:genetics; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D016133:Polymerase Chain Reaction; D011379:Prognosis; D000075142:RUNX1 Translocation Partner 1 Protein; D015996:Survival Rate
chemical_list: C107844:AML1-ETO fusion protein, human; C095514:CBFbeta-MYH11 fusion protein; D050676:Core Binding Factor Alpha 2 Subunit; D015514:Oncogene Proteins, Fusion; D000075142:RUNX1 Translocation Partner 1 Protein; C093476:promyelocytic leukemia-retinoic acid receptor alpha fusion oncoprotein