Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
8
pubmed:dateCreated
2002-9-27
pubmed:abstractText
The transcription factor C/EBPalpha is crucial for differentiation of mature granulocytes. Recently, different CEBPA gene mutations likely to induce differentiation arrest have been described in nearly 10% of patients with acute myeloid leukemia (AML). In the present study, we retrospectively analyzed the prognostic significance of CEBPA mutations in 135 AML patients (French-American-British [FAB]-M3 excluded). All patients were prospectively enrolled between 1990 and 1996 in a multicenter trial of the ALFA (Acute Leukemia French Association) Group (median age 45 years, median follow-up 5.7 years). Mutations were assessed using direct sequencing of the CEBPA gene. Twenty-two mutations were found in 15 (11%) of 135 patients tested. Twelve patients had at least one mutation located in the N-terminal part of the protein leading to the lack of expression of the full-length C/EBPalpha protein. CEBPA mutations were present only in patients belonging to the intermediate cytogenetic risk subgroup and associated with the FAB-M1 subtype (P =.02). FLT3 internal tandem duplication (ITD) was found in 5 of 15 CEBPA-mutated as compared with 30 of 119 CEBPA-nonmutated cases tested (P =.54). Presence of CEBPA mutations was identified as an independent good prognosis factor for outcome even after adjustment on cytogenetics and FLT3 status (estimated 5-year overall survival 53% vs 25%, P =.04). FLT3-ITD appeared to act as a major bad prognosis factor in patients with CEBPA-mutated AML. We thus propose a risk classification that includes in the favorable subgroup all patients from the intermediate subgroup displaying CEBPA mutations when not associated with FLT3-ITD.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Oct
pubmed:issn
0006-4971
pubmed:author
pubmed:issnType
Print
pubmed:day
15
pubmed:volume
100
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
2717-23
pubmed:dateRevised
2007-11-15
pubmed:meshHeading
pubmed-meshheading:12351377-Adolescent, pubmed-meshheading:12351377-Adult, pubmed-meshheading:12351377-Bone Marrow, pubmed-meshheading:12351377-CCAAT-Enhancer-Binding Protein-alpha, pubmed-meshheading:12351377-Female, pubmed-meshheading:12351377-Follow-Up Studies, pubmed-meshheading:12351377-France, pubmed-meshheading:12351377-Great Britain, pubmed-meshheading:12351377-Humans, pubmed-meshheading:12351377-Leukemia, Myeloid, Acute, pubmed-meshheading:12351377-Male, pubmed-meshheading:12351377-Middle Aged, pubmed-meshheading:12351377-Mutation, pubmed-meshheading:12351377-Prognosis, pubmed-meshheading:12351377-Sequence Deletion, pubmed-meshheading:12351377-Survival Rate, pubmed-meshheading:12351377-Time Factors, pubmed-meshheading:12351377-Treatment Outcome, pubmed-meshheading:12351377-United States
pubmed:year
2002
pubmed:articleTitle
Favorable prognostic significance of CEBPA mutations in patients with de novo acute myeloid leukemia: a study from the Acute Leukemia French Association (ALFA).
pubmed:affiliation
Département d'Hématologie and INSERM U524, Hôpital Claude Huriez, Lille, France. cpreudhomme@chru-lille.fr
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't