Table 1 Characteristics of T-ALL patients according to WT1 mutational status
From: Wilms tumor 1 (WT1) gene mutations in pediatric T-cell malignancies
Total | WT1 wild-type | WT1 mutant | P-value | |
|---|---|---|---|---|
No. (%) | No. (%) | No. (%) | ||
Patients | 143 (100%) | 128 (90%) | 15 (10%) | |
Age (years) | ||||
Median | 8.9 | 9.0 | 7.7 | 0.678 |
Range | 1.2–17.3 | 1.2–17.3 | 4.8–16.5 | |
Sex | ||||
Male | 107 (75%) | 96 (75%) | 11 (73%) | 1.0 |
Female | 36 (25%) | 32 (25%) | 4 (27%) | |
WBC count (10 9 /l) | ||||
Median | 54.6 | 54.6 | 45.5 | 0.886 |
Range | 0.2–872.0 | 0.2–872.0 | 1.0–415.0 | |
Mediastinal involvement | ||||
No | 79 (58%) | 71 (58%) | 8 (53%) | 0.785 |
Yes | 58 (42%) | 51 (42%) | 7 (47%) | |
Missing | 6 | 6 | 0 | |
Response to prephase | ||||
<1000 blasts per μl | 91 (64%) | 83 (65%) | 8 (53%) | 0.400 |
⩾1000 blasts per μl | 51 (36%) | 44 (35%) | 7 (47%) | |
Missing | 1 (1%) | 1 (1%) | 0 (0%) | |
Minimal residual disease (MRD) | ||||
<10−2 | 80 (83%) | 75 (84%) | 5 (71%) | 0.330 |
⩾10−2 | 16 (17%) | 14 (16%) | 2 (29%) | |
Absence of markers | 4 (3%) | 4 (3%) | 0 (0%) | |
Missing or ND | 43 (30%) | 35 (27%) | 8 (53%) | |
Very high risk (VHR) | ||||
No | 84 (61%) | 77 (63%) | 7 (47%) | 0.269 |
Yes | 54 (39%) | 46 (37%) | 8 (53%) | |
Missing | 5 (3%) | 5 (4%) | 0 (0%) | |
Immunophenotype (EGIL) | ||||
Early (TI/TII) | 37 (33%) | 31 (31%) | 6 (46%) | 0.353* |
Cortical (TIII) | 49 (44%) | 45 (46%) | 4 (31%) | |
Mature (TIV) | 26 (23%) | 23 (23%) | 3 (23%) | |
T-lineage (no further specified) | 31 (22%) | 29 (23%) | 2 (13%) | |
Oncogenic classifying alterations | ||||
SIL-TAL (+) | 20 (14%) | 20 (16%) | 0 (0%) | 0.130 |
HOX11/TLX1 (+) | 9 (6%) | 7 (5%) | 2 (13%) | 0.240 |
HOX11L2/TLX3 (+) | 31 (22%) | 23 (18%) | 8 (53%) | 0.004 |
CALM-AF10 (+) | 6/110 (5%) | 6/98 (6%) | 0/12 (0%) | 1.0 |
MLL rearrangement | 5 (3%) | 4 (3%) | 1 (7%) | 0.430 |
Other HOXA10 (+) | 3/70 (4%) | 2/64 (3%) | 1/6 (17%) | 0.492 |
Other genetic alterations | ||||
NUP214-ABL (+) | 9/129 (7%) | 7/115 (6%) | 2/14 (14%) | 0.253 |
MYB duplication (+) | 7/70 (10%) | 6/62 (10%) | 1/8 (12%) | 1.0 |
NOTCH1 mut and/or FBXW7 mut | 78/131 (60%) | 70/118 (59%) | 8/13 (62%) | 1.0 |
