Excellent prognosis of late relapses of ETV6/RUNX1-positive childhood acute lymphoblastic leukemia: lessons from the FRALLE 93 protocol.

Virginie Gandemer 1, * Sylvie Chevret 2 Arnaud Petit 3 Christiane Vermylen 4 Thierry Leblanc 3 Gerard Michel 5 Claudine Schmitt 6 Odile Lejars 7 Pascale Schneider 8 Francois Demeocq 9 Brigitte Bader-Meunier 10 Francoise Bernaudin 11 Yves Perel 12 Marie-Francoise Auclerc 2 Jean-Michel Cayuela 13 Guy Leverger 14 Andre Baruchel 3
* Auteur correspondant
1 Service de médecine de l'enfant et de l'adolescent
2 Biostatistique et épidemiologie clinique
3 Service d'hématologie et immunologie pédiatrique
4 Génétique Hématologique
5 Service d'hématologie pédiatrique
6 Service d'Hématologie Pédiatrique [CHRU Nancy]
7 Service d'hématologie pédiatrique
8 Service d'hémato-immuno-oncologie pédiatrique [Rouen]
9 Service d'hématologie pédiatrique
10 Service d'hématologie, immunologie biologiques et cytogénétique
11 Service d'hématologie pédiatrique
12 Service d'Hémato-oncologie Pédiatrique
13 Laboratoire central d'hématologie
14 Service d'hématologie-immunologie-oncologie pédiatrique
Abstract : Purpose. The prognosis of relapses of ETV6/RUNX1-positive acute lymphoblastic leukemia remains to be evaluated, particularly with regards to the frequency of late relapses. We performed a long term follow-up a retrospective study to address the outcome of ETV6/RUNX1-positive leukemia relapses.Design and Method. Among the 713 children tested for ETV6/RUNX1 enrolled into the FRALLE 93 protocol, 43 ETV6/RUNX1-positive patients relapsed (19.4%). Most were initially stratified as low or intermediate risk groups. Median follow-up after relapse was 54.2 months. All but three received a second-line salvage therapy and 16 underwent an allogeneic transplantation.Results. ETV6/RUNX1 greatly impacted on overall survival after relapse (3 year-survival= 64.7 % for positive versus 46.5 % for negative cases) (p= 0.007). The 5-year cumulative incidence of relapse was 19.4% and testes were more frequently involved in ETV6/RUNX1-positive relapses (p=0.04). 81.4 % were late relapses, early combined or isolated extramedullar relapses. The 5-year survival rate of ETV6-RUNX1-positive acute lymphoblastic leukemia relapses reached 80.6% when relapse occurred after 36 months (vs 34.9%). In univariate analysis, female gender was associated with a poor survival, whereas site of relapse, age at diagnosis, leukocytosis and consolidation strategy had no effect. In multivariate analysis, only the duration of first remission remained associated with outcome.Conclusions. We found an excellent outcome for ETV6/RUNX1-positive leukemia relapses occurring over 36 months post-diagnosis. Duration of first complete remission may thus be a guide to define the treatment strategy of ETV6/RUNX1-positive leukemia relapse.
Keywords : ETV6/RUNX1 childhood leukemia acute lymphoblastic prognosis relapse
Type de document :
Article dans une revue
Haematologica, Ferrata Storti Foundation, 2012, 97 (11), pp.1743-1750. 〈10.3324/haematol.2011.059584〉
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http://www.hal.inserm.fr/inserm-00700077
Contributeur : Hervé De Villemeur <>
Soumis le : mardi 22 mai 2012 - 12:04:32
Dernière modification le : vendredi 16 novembre 2018 - 02:09:52
Document(s) archivé(s) le : jeudi 23 août 2012 - 02:27:43

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Virginie Gandemer, Sylvie Chevret, Arnaud Petit, Christiane Vermylen, Thierry Leblanc, et al.. Excellent prognosis of late relapses of ETV6/RUNX1-positive childhood acute lymphoblastic leukemia: lessons from the FRALLE 93 protocol.. Haematologica, Ferrata Storti Foundation, 2012, 97 (11), pp.1743-1750. 〈10.3324/haematol.2011.059584〉. 〈inserm-00700077〉

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