Source:http://linkedlifedata.com/resource/pubmed/id/12351377
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
8
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pubmed:dateCreated |
2002-9-27
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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.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
AIM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Oct
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pubmed:issn |
0006-4971
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pubmed:author |
pubmed-author:ALFA Group,
pubmed-author:BoisselNicolasN,
pubmed-author:CastaigneSylvieS,
pubmed-author:CayuelaJean-MichelJM,
pubmed-author:DombretHervéH,
pubmed-author:FenauxPierreP,
pubmed-author:LamandinCharlotteC,
pubmed-author:PreudhommeClaudeC,
pubmed-author:RaffouxEmmanuelE,
pubmed-author:SagotChristopheC,
pubmed-author:ThomasXavierX,
pubmed-author:TigaudIsabelleI,
pubmed-author:de BottonStéphaneS
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pubmed:issnType |
Print
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pubmed:day |
15
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pubmed:volume |
100
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
2717-23
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pubmed:dateRevised |
2007-11-15
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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
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pubmed:year |
2002
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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).
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pubmed:affiliation |
Département d'Hématologie and INSERM U524, Hôpital Claude Huriez, Lille, France. cpreudhomme@chru-lille.fr
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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