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An intragenic ERG deletion is a marker of an oncogenic subtype of B-cell precursor acute lymphoblastic leukemia with a favorable outcome despite frequent IKZF1 deletions.
Clappier, E; Auclerc, M F; Rapion, J; Bakkus, M; Caye, A; Khemiri, A; Giroux, C; Hernandez, L; Kabongo, E; Savola, S; Leblanc, T; Yakouben, K; Plat, G; Costa, V; Ferster, A; Girard, S; Fenneteau, O; Cayuela, J M; Sigaux, F; Dastugue, N; Suciu, S; Benoit, Y; Bertrand, Y; Soulier, J; Cavé, H.
Afiliación
  • Clappier E; 1] U944 INSERM and Hematology laboratory, St-Louis Hospital, APHP, Paris, France [2] Department of Genetics, Robert Debré Hospital, APHP, Paris, France [3] Hematology University Institute, University Paris-Diderot, Paris, France.
  • Auclerc MF; 1] U944 INSERM and Hematology laboratory, St-Louis Hospital, APHP, Paris, France [2] Department of Pediatric Hematology, St-Louis Hospital, APHP, Paris, France.
  • Rapion J; EORTC Headquarters, Brussels, Belgium.
  • Bakkus M; Molecular Hematology Laboratory, UZ Brussels, Brussels, Belgium.
  • Caye A; Department of Genetics, Robert Debré Hospital, APHP, Paris, France.
  • Khemiri A; Department of Genetics, Robert Debré Hospital, APHP, Paris, France.
  • Giroux C; Department of Genetics, Robert Debré Hospital, APHP, Paris, France.
  • Hernandez L; U944 INSERM and Hematology laboratory, St-Louis Hospital, APHP, Paris, France.
  • Kabongo E; Molecular Hematology Laboratory, UZ Brussels, Brussels, Belgium.
  • Savola S; MRC-Holland, Amsterdam, The Netherlands.
  • Leblanc T; Department of Pediatric Hematology, St-Louis Hospital, APHP, Paris, France.
  • Yakouben K; Department of Pediatric Hematology, Robert-Debré Hospital, APHP, Paris, France.
  • Plat G; Department of Pediatric Onco-Hematology, University Hospital Purpan, Toulouse, France.
  • Costa V; Department of Pediatrics, Portuguese Oncology Institute, Porto, Portugal.
  • Ferster A; Department of Pediatric Onco-Hematology, Children's University Hospital Reine Fabiola, Brussels, Belgium.
  • Girard S; Hematology Laboratory, IHOP, Lyon, France.
  • Fenneteau O; Hematology Laboratory, Robert Debré Hospital, APHP, Paris, France.
  • Cayuela JM; 1] U944 INSERM and Hematology laboratory, St-Louis Hospital, APHP, Paris, France [2] Hematology University Institute, University Paris-Diderot, Paris, France.
  • Sigaux F; 1] U944 INSERM and Hematology laboratory, St-Louis Hospital, APHP, Paris, France [2] Hematology University Institute, University Paris-Diderot, Paris, France.
  • Dastugue N; Hematology Laboratory, University Hospital Purpan, Toulouse, France.
  • Suciu S; EORTC Headquarters, Brussels, Belgium.
  • Benoit Y; Department of Pediatric Hematology-Oncology, Ghent University Hospital, Ghent, Belgium.
  • Bertrand Y; Department of Pediatric Hematology, IHOP and Claude Bernard University, Lyon, France.
  • Soulier J; 1] U944 INSERM and Hematology laboratory, St-Louis Hospital, APHP, Paris, France [2] Hematology University Institute, University Paris-Diderot, Paris, France.
  • Cavé H; 1] Department of Genetics, Robert Debré Hospital, APHP, Paris, France [2] Hematology University Institute, University Paris-Diderot, Paris, France.
Leukemia ; 28(1): 70-7, 2014 Jan.
Article en En | MEDLINE | ID: mdl-24064621
Oncogenic subtypes in childhood B-cell precursor acute lymphoblastic leukemia (BCP-ALL) are used for risk stratification. However, a significant number of BCP-ALL patients are still genetically unassigned. Using array-comparative genomic hybridization in a selected BCP-ALL cohort, we characterized a recurrent V(D)J-mediated intragenic deletion of the ERG gene (ERG(del)). A breakpoint-specific PCR assay was designed and used to screen an independent non-selected cohort of 897 children aged 1-17 years treated for BCP-ALL in the EORTC-CLG 58951 trial. ERG(del) was found in 29/897 patients (3.2%) and was mutually exclusive of known classifying genetic lesions, suggesting that it characterized a distinct leukemia entity. ERG(del) was associated with higher age (median 7.0 vs. 4.0 years, P=0.004), aberrant CD2 expression (43.5% vs. 3.7%, P<0.001) and frequent IKZF1 Δ4-7 deletions (37.9% vs. 5.3%, P<0.001). However, ERG(del) patients had a very good outcome, with an 8-year event-free survival (8-y EFS) and an 8-year overall survival of 86.4% and 95.6%, respectively, suggesting that the IKZF1 deletion had no impact on prognosis in this genetic subtype. Accordingly, within patients with an IKZF1 Δ4-7 deletion, those with ERG(del) had a better outcome (8-y EFS: 85.7% vs. 51.3%; hazard ratio: 0.16; 95% confidence interval: 0.02-1.20; P=0.04). These findings have implications for further stratification including IKZF1 status.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Leucemia-Linfoma Linfoblástico de Células Precursoras B / Transactivadores / Eliminación de Gen / Factor de Transcripción Ikaros Tipo de estudio: Prognostic_studies Límite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male Idioma: En Revista: Leukemia Asunto de la revista: HEMATOLOGIA / NEOPLASIAS Año: 2014 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Reino Unido
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Leucemia-Linfoma Linfoblástico de Células Precursoras B / Transactivadores / Eliminación de Gen / Factor de Transcripción Ikaros Tipo de estudio: Prognostic_studies Límite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male Idioma: En Revista: Leukemia Asunto de la revista: HEMATOLOGIA / NEOPLASIAS Año: 2014 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Reino Unido