Clinical, immunophenotypic, and genotypic characteristics of adult T-ALL as a function of NOTCH1/FBXW7 status
| . | Total (%) . | NOTCH1 mutation . | FBXW7 mutation . | NOTCH1 and/or FBXW7 mutation . | |||
|---|---|---|---|---|---|---|---|
| Mutant, n (%) . | Wild-type, n (%) . | Mutant, n (%) . | Wild-type, n (%) . | Mutant, n (%) . | Wild-type, n (%) . | ||
| 141 | 88 (62) | 53 (38) | 34 (24) | 107 (76) | 101 (72) | 40 (28) | |
| TCR subsets analyzed | |||||||
| Immature | 42 | 25 (60) | 17 (40) | 6 (14) | 36 (86) | 27 (64) | 15 (36) |
| Pre-αβ | 44 | 30 (68) | 14 (32) | 13 (30) | 31 (70) | 35 (80) | 9 (20) |
| TCR+ | 29 | 13 (45) | 15 (53) | 7 (24) | 22 (76) | 19 (65) | 10 (34) |
| ND | 26 | 19 (73) | 7 (27) | 8 (31) | 18 (70) | 20 (77) | 6 (23) |
| EGIL | |||||||
| 1-2 | 43 | 25 (58) | 18 (42) | 6 (14) | 37 (86) | 27 (63) | 16 (37) |
| 3 | 73 | 52 (71) | 21 (29) | 24 (33) | 49 (67) | 59 (81) | 14 (19) |
| 4 | 15 | 7 (47) | 8 (53) | 4 (27) | 11 (73) | 11 (73) | 4 (27) |
| ND | 10 | 4 (40) | 6 (60) | 0 (0) | 10 (100) | 4 (40) | 6 (60) |
| Genotype subsets analyzed | |||||||
| CALM-AF10 | 7 | 3 (43) | 4 (57) | 4 (57) | 3 (43) | 5 (71) | 2 (29) |
| SIL-TAL1 | 10 | 4 (40) | 6 (60) | 1 (10) | 9 (90) | 5 (50) | 5 (50) |
| HOX11 | 21 | 18 (86) | 3 (14) | 5 (24) | 16 (76) | 20 (95) | 1 (5) |
| HOX11L2 | 13 | 7 (54) | 6 (46) | 4 (31) | 9 (69) | 10 (77) | 3 (23) |
| None of above | 76 | 44 (58) | 32 (42) | 17 (22) | 59 (78) | 48 (63) | 28 (37) |
| ND | 14 | 12 (86) | 2 (14) | 3 (21) | 11 (79) | 13 (93) | 1 (7) |
| Clinical subsets analyzed | |||||||
| Median age, y | 28 | 28 | 28 | 28 | 31 | 27 | 29 |
| Age >35 | 46 (33) | 30 (34) | 16 (30) | 10 (29) | 36 (34) | 33 (33) | 13 (33) |
| Median WBC | 54 | 51 | 94 | 59 | 94 | 51 | 104 |
| WBC >100g/L | 52 (37) | 28 (31) | 24 (45) | 7 (21) | 45 (42) | 31 (31) | 21 (53) |
| High-risk MRC-ECOG | 85 (60) | 50 (57) | 35 (66) | 16 (47) | 69 (64) | 56 (56) | 29 (73) |
| Mediastinal involvement | 66 (47) | 43 (48) | 23 (43) | 20 (59) | 46 (43) | 51 (52) | 15 (38) |
| CNS involvement | 12 (9) | 4 (5) | 8 (15) | 2 (6) | 10 (9) | 6 (6) | 6 (15) |
| . | Total (%) . | NOTCH1 mutation . | FBXW7 mutation . | NOTCH1 and/or FBXW7 mutation . | |||
|---|---|---|---|---|---|---|---|
| Mutant, n (%) . | Wild-type, n (%) . | Mutant, n (%) . | Wild-type, n (%) . | Mutant, n (%) . | Wild-type, n (%) . | ||
| 141 | 88 (62) | 53 (38) | 34 (24) | 107 (76) | 101 (72) | 40 (28) | |
| TCR subsets analyzed | |||||||
| Immature | 42 | 25 (60) | 17 (40) | 6 (14) | 36 (86) | 27 (64) | 15 (36) |
| Pre-αβ | 44 | 30 (68) | 14 (32) | 13 (30) | 31 (70) | 35 (80) | 9 (20) |
| TCR+ | 29 | 13 (45) | 15 (53) | 7 (24) | 22 (76) | 19 (65) | 10 (34) |
| ND | 26 | 19 (73) | 7 (27) | 8 (31) | 18 (70) | 20 (77) | 6 (23) |
| EGIL | |||||||
| 1-2 | 43 | 25 (58) | 18 (42) | 6 (14) | 37 (86) | 27 (63) | 16 (37) |
| 3 | 73 | 52 (71) | 21 (29) | 24 (33) | 49 (67) | 59 (81) | 14 (19) |
| 4 | 15 | 7 (47) | 8 (53) | 4 (27) | 11 (73) | 11 (73) | 4 (27) |
| ND | 10 | 4 (40) | 6 (60) | 0 (0) | 10 (100) | 4 (40) | 6 (60) |
| Genotype subsets analyzed | |||||||
| CALM-AF10 | 7 | 3 (43) | 4 (57) | 4 (57) | 3 (43) | 5 (71) | 2 (29) |
| SIL-TAL1 | 10 | 4 (40) | 6 (60) | 1 (10) | 9 (90) | 5 (50) | 5 (50) |
| HOX11 | 21 | 18 (86) | 3 (14) | 5 (24) | 16 (76) | 20 (95) | 1 (5) |
| HOX11L2 | 13 | 7 (54) | 6 (46) | 4 (31) | 9 (69) | 10 (77) | 3 (23) |
| None of above | 76 | 44 (58) | 32 (42) | 17 (22) | 59 (78) | 48 (63) | 28 (37) |
| ND | 14 | 12 (86) | 2 (14) | 3 (21) | 11 (79) | 13 (93) | 1 (7) |
| Clinical subsets analyzed | |||||||
| Median age, y | 28 | 28 | 28 | 28 | 31 | 27 | 29 |
| Age >35 | 46 (33) | 30 (34) | 16 (30) | 10 (29) | 36 (34) | 33 (33) | 13 (33) |
| Median WBC | 54 | 51 | 94 | 59 | 94 | 51 | 104 |
| WBC >100g/L | 52 (37) | 28 (31) | 24 (45) | 7 (21) | 45 (42) | 31 (31) | 21 (53) |
| High-risk MRC-ECOG | 85 (60) | 50 (57) | 35 (66) | 16 (47) | 69 (64) | 56 (56) | 29 (73) |
| Mediastinal involvement | 66 (47) | 43 (48) | 23 (43) | 20 (59) | 46 (43) | 51 (52) | 15 (38) |
| CNS involvement | 12 (9) | 4 (5) | 8 (15) | 2 (6) | 10 (9) | 6 (6) | 6 (15) |
No statistically significant differences were observed.