Clinico-biological features of T-cell acute lymphoblastic leukemia with fusion proteins.
(2022) Blood cancer journal — Vol. 12, n° 1, p. 14 [1-8] (2022)
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- Authors
Alcantara, Marion 
Asnafi, Vahid
- Abstract
- T-cell acute lymphoblastic leukemias (T-ALL) represent 15% of pediatric and 25% of adult ALL. Since they have a particularly poor outcome in relapsed/refractory cases, identifying prognosis factors at diagnosis is crucial to adapting treatment for high-risk patients. Unlike acute myeloid leukemia and BCP ALL, chromosomal rearrangements leading to chimeric fusion-proteins with strong prognosis impact are sparsely reported in T-ALL. To address this issue an RT-MPLA assay was applied to a consecutive series of 522 adult and pediatric T-ALLs and identified a fusion transcript in 20% of cases. PICALM-MLLT10 (4%, n = 23), NUP214-ABL1 (3%, n = 19) and SET-NUP214 (3%, n = 18) were the most frequent. The clinico-biological characteristics linked to fusion transcripts in a subset of 235 patients (138 adults in the GRAALL2003/05 trials and 97 children from the FRALLE2000 trial) were analyzed to identify their prognosis impact. Patients with HOXA trans-deregulated T-ALLs with MLLT10, KMT2A and SET fusion transcripts (17%, 39/235) had a worse prognosis with a 5-year EFS of 35.7% vs 63.7% (HR = 1.63; p = 0.04) and a trend for a higher cumulative incidence of relapse (5-year CIR = 45.7% vs 25.2%, HR = 1.6; p = 0.11). Fusion transcripts status in T-ALL can be robustly identified by RT-MLPA, facilitating risk adapted treatment strategies for high-risk patients.
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Steimlé, T., Dourthe, M.-E., Alcantara, M., Touzart, A., Simonin, M., Mondesir, J., Lhermitte, L., Bond, J., Graux, C., Grardel, N., Cayuela, J.-M., Arnoux, I., Gandemer, V., Balsat, M., Vey, N., Macintyre, E., Ifrah, N., Dombret, H., Petit, A., et al. (2022). Clinico-biological features of T-cell acute lymphoblastic leukemia with fusion proteins. Blood cancer journal, 12(1), 14 [1-8]. https://doi.org/10.1038/s41408-022-00613-9 (Original work published 2022)