SET-NUP214 is a recurrent gammadelta lineage-specific fusion transcript associated with corticosteroid/chemotherapy resistance in adult T-ALL.

Date publication

mars 2014

Auteurs

Membres identifiés du Cancéropôle Est :
Dr CAILLOT Denis , Dr LIOURE Bruno

Tous les auteurs :
Ben Abdelali R, Roggy A, Leguay T, Cieslak A, Renneville A, Touzart A, Banos A, Randriamalala E, Caillot D, Lioure B, Devidas A, Mossafa H, Preudhomme C, Ifrah N, Dombret H, Macintyre E, Asnafi V

Lien Pubmed

https://www.ncbi.nlm.nih.gov/pubmed/24449214

Résumé

The SET-NUP214 (TAF1/CAN) fusion gene is a rare genetic event in T-cell acute lymphoblastic leukemia (T-ALL). Eleven (6%) of 196 T-ALL patients enrolled in the French Group for Research on Adult Acute Lymphoblastic Leukemia (GRAALL) 2003 and 2005 trials harbored a SET-NUP214 transcript. SET-NUP214-positive patients were predominantly (10 [91%] of 11) T-cell receptor (TCR)-negative and strikingly associated with TCRgammadelta lineage T-ALLs, as defined by expression of TCRgammadelta, TCRdelta and/or TCRgamma rearrangements but no complete TCRbeta variable diversity joining rearrangement in surface CD3/TCR-negative cases. When compared with SET-NUP214-negative patients, SET-NUP214-positive patients showed a significantly higher rate of corticosteroid resistance (91% vs 44%; P = .003) and chemotherapy resistance (100% vs 44%; P = .0001). All SET-NUP214-positive patients but one achieved complete remission, and 9 were allografted. Despite the poor early-treatment sensitivity, the outcome of SET-NUP214-positive patients was similar to that of SET-NUP214-negative patients.

Référence

Blood. 2014 Mar 20;123(12):1860-3