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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 (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 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 Citing Articles via Google Scholar Google Scholar Articles by Ho, P. A. Articles by Meshinchi, S. Search for Related Content PubMed PubMed Citation Articles by Ho, P. A. Articles by Meshinchi, S. Related Collections Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted October 24, 2008 Accepted February 27, 2009 Prevalence and prognostic implications of CEBPA mutations in pediatric AML: a report from the Children's Oncology Group Phoenix A. Ho*, Todd A. Alonzo, Robert B. Gerbing, Jessica Pollard, Derek L. Stirewalt, Craig Hurwitz, Nyla A. Heerema, Betsy Hirsch, Susana C. Raimondi, Beverly Lange, Janet L. Franklin, Jerald P. Radich, and Soheil Meshinchi Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA, United States Department of Biostatistics, University of Southern California, Los Angeles, CA, United States Children's Oncology Group, Arcadia, CA, United States Department of Pediatrics, University of Washington, Seattle, WA, United States Department of Pediatric Oncology, Maine Children's Cancer Program at Maine Medial Center, Portland, OR, United States Department of Pathology, The Ohio State University, Columbus, OH, United States Department of Laboratory Medicine and Pathology, University of Minnesota Cancer Center, Minneapolis, MN, United States Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN, United States Department of Oncology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Department of Hematology/Oncology, Children's Hospital Los Angeles, Los Angeles, CA, United States * Corresponding author; email: pho{at}fhcrc.org. The transcription factor C/EBP regulates terminal granulocytic differentiation. Functional CEBPA mutations have been associated with improved outcome in adult AML. We evaluated the prevalence and prognostic significance of CEBPA mutations in 847 children with AML treated on three consecutive pediatric trials (CCG-2941, CCG-2961, and COG-AAML03P1). Two types of CEBPA mutations-N-terminal truncating mutations and in-frame bZip-domain mutations- were detected in 38/847 (4.5%) patients tested; 31/38 (82%) patients with mutations harbored both mutation types. The presence of mutations 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% vs. 38% (p=0.015) with a cumulative incidence of relapse from complete remission of 13% vs. 44% (p=0.007) for those with and without CEBPA mutations. The presence of CEBPA mutations was an independent prognostic factor for improved outcome (HR 0.24, p=0.047). 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 www.clinicaltrials.gov under NCT00002798 and NCT00070174. 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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