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Blood, 25 June 2009, Vol. 113, No. 26, pp. 6558-6566. Prepublished online as a Blood First Edition Paper on March 20, 2009; DOI 10.1182/blood-2008-10-184747. This Article Full Text Full Text (PDF) Supplemental Appendix All Versions of this Article: blood-2008-10-184747v1 113/26/6558    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Google Scholar Articles by Ho, P. A. Articles by Meshinchi, S. PubMed PubMed Citation Articles by Ho, P. A. Articles by Meshinchi, S. Related Collections Free Research Articles Myeloid Neoplasia Clinical Trials and Observations Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS Prevalence and prognostic implications of CEBPA mutations in pediatric acute myeloid leukemia (AML): a report from the Children's Oncology Group Phoenix A. Ho1,2, Todd A. Alonzo3,4, Robert B. Gerbing4, Jessica Pollard1,2,4, Derek L. Stirewalt1, Craig Hurwitz5, Nyla A. Heerema6, Betsy Hirsch7, Susana C. Raimondi8, Beverly Lange9, Janet L. Franklin10, Jerald P. Radich1, and Soheil Meshinchi1,2 1 Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA; 2 Department of Pediatrics, University of Washington, Seattle; 3 Department of Biostatistics, University of Southern California, Los Angeles; 4 Children's Oncology Group, Arcadia, CA; 5 Department of Pediatric Oncology, Maine Children's Cancer Program at Maine Medical Center, Portland; 6 Department of Pathology, The Ohio State University, Columbus; 7 Department of Laboratory Medicine and Pathology, University of Minnesota Cancer Center, Minneapolis; 8 Department of Pathology, St Jude Children's Research Hospital, Memphis, TN; 9 Department of Oncology, Children's Hospital of Philadelphia, PA; and 10 Department of Hematology/Oncology, Children's Hospital Los Angeles, CA CEBPA mutations have been associated with improved outcome in adult acute myeloid leukemia (AML). We evaluated the prevalence and prognostic significance of CEBPA mutations in 847 children with AML treated on 3 consecutive pediatric trials. Two types of CEBPA mutations—N-terminal truncating mutations and in-frame bZip-domain mutations—were detected in 38 (4.5%) of 847 patients tested; 31 (82%) of 38 patients with mutations harbored both mutation types. Mutation status was correlated with laboratory and clinical characteristics and clinical outcome. CEBPA mutations were significantly more common in older patients, patients with FAB M1 or M2, and patients with normal karyotype. Mutations did not occur in patients with either favorable or unfavorable cytogenetics. Actuarial event-free survival at 5 years was 70% versus 38% (P = .015) with a cumulative incidence of relapse from complete remission of 13% versus 44% (P = .007) for those with and without CEBPA mutations. The presence of CEBPA mutations was an independent prognostic factor for improved outcome (HR = 0.24, P = .047). As CEBPA mutations are associated with lower relapse rate and improved survival, CEBPA mutation analysis needs to be incorporated into initial screening for risk identification and therapy allocation at diagnosis. The clinical trials in this study are registered at http://www.clinicaltrials.gov under NCT00002798 [ClinicalTrials.gov] and NCT00070174 [ClinicalTrials.gov] . CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: CEBPA resembles Roman god Janus Iris H. I. M. Hollink, Marry M. van den Heuvel-Eibrink, and Christian Michel Zwaan Blood 2009 113: 6501-6502. [Full Text] [PDF] This article has been cited by other articles: I. H. I. M. Hollink, M. M. van den Heuvel-Eibrink, and C. M. Zwaan CEBPA resembles Roman god Janus Blood, June 25, 2009; 113(26): 6501 - 6502. [Full Text] [PDF]

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