title: The clinical spectrum of adult acute myeloid leukaemia associated with core binding factor translocations.
abstract: To better understand the spectrum of adult acute myeloid leukaemia (AML) associated with core binding factor (CBF) translocations, 370 patients with newly diagnosed CBF-associated AML were analysed. Patients' age ranged from 16-83 years (median 39 years) with a slight male predominance (55%); 53% had inv(16); 47% had t(8;21). Patients with t(8;21) tended to be younger (P = 0.056), have lower peripheral blood white cell counts (P < 0.0001) and were more likely to have additional cytogenetic abnormalities (P < 0.0001). Loss of sex chromosome, del(9q) and complex abnormalities were more common among patients with t(8;21), while +22 and +21 were more common with inv(16). Overall, 87% [95% confidence interval (CI) 83-90%] of patients achieved complete response (CR) with no difference between t(8;21) and inv(16); however, the CR rate was lower in older patients due to increased resistant disease and early deaths. Ten-year overall survival (OS) was 44% (95% CI 39-50%) and, in multivariate analysis, was shorter with increasing age (P < 0.0001), increased peripheral blast percentage (P = 0.0006), in patients with complex cytogenetic abnormalities in addition to the CBF translocation (P = 0.021), and in patients with t(8;21) (P = 0.025). OS was superior in patients who received regimens with high-dose cytarabine, a combination of fludarabine and intermediate-dose cytarabine, or haematopoietic cell transplantation.
authors.affiliation: Southwest Oncology Group Statistical Center, Seattle, WA, USA. fappelb@fhcrc.org
authors.forename: Frederick R
authors.initials: FR
authors.lastname: Appelbaum
authors.forename: Kenneth J
authors.initials: KJ
authors.lastname: Kopecky
authors.forename: Martin S
authors.initials: MS
authors.lastname: Tallman
authors.forename: Marilyn L
authors.initials: ML
authors.lastname: Slovak
authors.forename: Holly M
authors.initials: HM
authors.lastname: Gundacker
authors.forename: Haesook T
authors.initials: HT
authors.lastname: Kim
authors.forename: Gordon W
authors.initials: GW
authors.lastname: Dewald
authors.forename: Hagop M
authors.initials: HM
authors.lastname: Kantarjian
authors.forename: Sherry R
authors.initials: SR
authors.lastname: Pierce
authors.forename: Elihu H
authors.initials: EH
authors.lastname: Estey
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002869:Chromosome Aberrations; D050660:Core Binding Factors / Q000235:genetics*; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007951:Leukemia, Myeloid / Q000097:blood / Q000188:drug therapy / Q000235:genetics*; D007958:Leukocyte Count; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D014178:Translocation, Genetic; D016896:Treatment Outcome
chemical_list: D050660:Core Binding Factors