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Prepublished online as a Blood First Edition Paper on September 22, 2003; DOI 10.1182/blood-2003-05-1495.

Submitted May 12, 2003
Accepted August 28, 2003
Clinical significance of HOX11L2 expression linked to t(5;14)(q35;q32), of HOX11 expression, and of SIL-TAL fusion in childhood T-cell malignancies: results of EORTC studies 58881 and 58951
Helene Cave*, Stefan Suciu, Claude Preudhomme, Bruce Poppe, Alain Robert, Anne Uyttebroeck, Michele Malet, Patrick Boutard, Yves Benoit, Laurent Mauvieux, Patrick Lutz, Francoise Mechninaud, Nathalie Grardel, Francoise Mazingue, Madeleine Dupont, Genevieve Margueritte, Marie-Pierre Pages, Yves Bertrand, Emmanuel Plouvier, Ghislaine Brunie, Christian Bastard, Dominique Plantaz, Isabel Vande Velde, Anne Hagemeijer, Frank Speleman, Michel Lessard, Jacques Otten, Etienne Vilmer, and Nicole Dastugue
Laboratoire de Biochimie Genetique, Hopital Robert Debre (AP-HP), Paris, France; Unite 417, INSERM, Paris, France
Data Center, EORTC, Brussels, Belgium
Laboratoire d'Hematologie A, Hopital Calmette, Lille, France
Universitair Ziekenhuis Ghent, Ghent, Belgium
Unite d'Hematologie Infantile, Hopital Purpan, Toulouse, France
U.Z Gasthuisberg, Leuven, Belgium
Unite d'Onco-Hematologie Pediatrique, CHU, Caen, France
Hopital universitaire Hautepierre, Strasbourg, France
CHR Hotel Dieu, Nantes, France
CHR de Lille, Lille, France
Hopital Arnaud de Villeneuve, Montpellier, France
Hopital Debrousse, Lyon, France
CHR de Besancon - Hopital Saint Jacques, Besancon, France
Centre Henri Becquerel, Rouen, France
CHU de Grenoble, La Tronche, France
Akademisch Ziekenhuis-VUB, Brussels, Belgium
Service d'Immuno-Hematologie Pediatrique, Hopital Robert Debre (AP-HP), Paris, France
Laboratoire d'Hematologie, Hopital Purpan, Toulouse, France
* Corresponding author; email: cave{at}infobiogen.fr.
In a series of 153 children with T-cell malignancies enrolled in 2 consecutive EORTC trials, we assessed the HOX11L2 expression and/or the presence of a t(5;14)(q35;q32). Additionally, in 138 of these patients, HOX11 expression and SIL-TAL rearrangement were also assessed. These alterations were mutually exclusive and their frequency was 23% (n=35), 7% (n=10) and 12% (n=17) respectively. HOX11L2/t(5;14) positivity was more frequent in ALL with cortical T- immunophenotype and in children between 6 and 9 years of age. In contrast with previously reported data, HOX11L2/t(5;14) positive and negative patients were comparable with regard to clinical outcome as well as to the response to a 7-day prephase treatment or to residual disease at completion of induction therapy. The 3-year event-free survival (EFS) rate (±SE%) for HOX11L2/t(5;14) positive and negative patients was 75.5% (±8.1%) and 68.3% (±5.0%) respectively; the hazard ratio was 0.84 [95% confidence interval (0.40, 1.80)]. Patients with HOX11-high expression and those with SIL-TAL fusion had low levels of residual disease at the end of induction and a favorable prognosis: the 3-year EFS rate was 83.3% (±8.5%) and 75.3% (±12.6%) respectively. The results obtained in HOX11L2/t(5;14) patients in this study do not confirm the unfavorable prognosis reported in previous studies.

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