Clinicobiologic characteristics of adult and pediatric patients with T-ALL (GRAALL and FRALLE protocols) according to PTPN2 status
| . | PTPN2 del, n = 33 (8%)* . | PTPN2 WT, n = 397 (92%) . | Total, N = 430 (100%) . | P . |
|---|---|---|---|---|
| Clinical subsets analyzed | ||||
| Male, n (%) | 22/33 (67) | 306/397 (77) | 328/430 (76) | .2 |
| Age, median (range), y | 27.4 (4.3-57.0) | 16.8 (1.1-59.1) | 17.5 (1.1-59.1) | .02 |
| WBC, median (range) | 48.1 (4.1-574) | 57.8 (0.3-980) | 57.7 (0.3-980) | .2 |
| CNS involvement (%) | 5/33 (15) | 37/394 (9) | 42/427 (10) | .3 |
| TCR status (available)† | 26/33 | 277/397 | 303/430 | |
| Immature (IM0, IMD, IMG), n (%) | 2/26 (8) | 64/277 (23) | 66/303 (22) | .08 |
| αβ lineage (IMB, pre-αβ, TCR αβ) , n (%) | 23/26 (88) | 174/277 (63) | 197/303 (65) | .009 |
| γδ lineage (TCR γδ), n (%) | 1/26 (4) | 39/277 (14) | 40/303 (13) | .2 |
| Oncogenetics, n (%)† | ||||
| TLX1 | 13/27 (48) | 39/308 (13) | 52/335 (15) | <.0001 |
| TLX3 | 9/27 (33) | 52/308 (17) | 61/335 (18) | .06 |
| SIL-TAL1 | 1/27 (4) | 37/308 (18) | 38/335 (17) | .3 |
| NUP214-ABL | 5/24 (21) | 17/257 (7) | 22/281 (8) | .03 |
| PTEN-deleted/mutated | 0/33 (0) | 55/396 (14) | 55/429 (13) | .01 |
| NOTCH1/FBXW7 mutated | 28/33 (85) | 250/397 (63) | 278/430 (65) | .01 |
| RAS mutated | 3/33 (9) | 36/397 (9) | 39/430 (9) | 1 |
| Treatment response, n (%)† | ||||
| Corticosensitivity | 25/32 (78) | 217/388 (56) | 242/420 (58) | .015 |
| CR | 32/33 (97) | 374/397 (94) | 406/430 (94) | 1 |
| MRD1 ≥ 10−4‡ | 9/20 (45) | 102/274 (37) | 111/294 (38) | .5 |
| . | PTPN2 del, n = 33 (8%)* . | PTPN2 WT, n = 397 (92%) . | Total, N = 430 (100%) . | P . |
|---|---|---|---|---|
| Clinical subsets analyzed | ||||
| Male, n (%) | 22/33 (67) | 306/397 (77) | 328/430 (76) | .2 |
| Age, median (range), y | 27.4 (4.3-57.0) | 16.8 (1.1-59.1) | 17.5 (1.1-59.1) | .02 |
| WBC, median (range) | 48.1 (4.1-574) | 57.8 (0.3-980) | 57.7 (0.3-980) | .2 |
| CNS involvement (%) | 5/33 (15) | 37/394 (9) | 42/427 (10) | .3 |
| TCR status (available)† | 26/33 | 277/397 | 303/430 | |
| Immature (IM0, IMD, IMG), n (%) | 2/26 (8) | 64/277 (23) | 66/303 (22) | .08 |
| αβ lineage (IMB, pre-αβ, TCR αβ) , n (%) | 23/26 (88) | 174/277 (63) | 197/303 (65) | .009 |
| γδ lineage (TCR γδ), n (%) | 1/26 (4) | 39/277 (14) | 40/303 (13) | .2 |
| Oncogenetics, n (%)† | ||||
| TLX1 | 13/27 (48) | 39/308 (13) | 52/335 (15) | <.0001 |
| TLX3 | 9/27 (33) | 52/308 (17) | 61/335 (18) | .06 |
| SIL-TAL1 | 1/27 (4) | 37/308 (18) | 38/335 (17) | .3 |
| NUP214-ABL | 5/24 (21) | 17/257 (7) | 22/281 (8) | .03 |
| PTEN-deleted/mutated | 0/33 (0) | 55/396 (14) | 55/429 (13) | .01 |
| NOTCH1/FBXW7 mutated | 28/33 (85) | 250/397 (63) | 278/430 (65) | .01 |
| RAS mutated | 3/33 (9) | 36/397 (9) | 39/430 (9) | 1 |
| Treatment response, n (%)† | ||||
| Corticosensitivity | 25/32 (78) | 217/388 (56) | 242/420 (58) | .015 |
| CR | 32/33 (97) | 374/397 (94) | 406/430 (94) | 1 |
| MRD1 ≥ 10−4‡ | 9/20 (45) | 102/274 (37) | 111/294 (38) | .5 |
Comparison of the clinicobiologic characteristics of PTPN2-deleted and wild-type T-ALL patients in the whole cohort. TCR status and oncogenetics were determined as previously described.4,21,23 Bold P values are statistically significant (P < .05).
CNS, central nervous system; CR, complete remission; del, deleted; IM0, no TCR rearrangement; IMB, VDJ rearrangement of TCRβ is observed; IMD, only TCRδ rearrangement is observed; IMG, both TCRδ and TCRγ rearrangements are observed; WBC, white blood cell count; WT, wild-type.
Monoallelic 16/33; biallelic 17/33.
Detailed data in supplemental Figure 3.
MRD was centrally assessed by real-time quantitative allele-specific oligonucleotide polymerase chain reaction and interpreted according to EuroMRD group guidelines.34,35