Table 2.
Features and Treatment Outcome of ETV6::RUNX1 Acute Lymphoblastic Leukemia
| ETV6::RUNX1 | All Protocols | MRD-guided Protocols Only | Non-MRD-guided Protocols Only | ||||||
|---|---|---|---|---|---|---|---|---|---|
| IKZF1 | IKZF1 | IKZF1 | |||||||
| Deleted | Wild-type | Deleted | Wild-type | Deleted | Wild-type | ||||
| n = 939 | n = 26 | n = 913 | P-value | n = 14 | n = 632 | P-value | n = 12 | n = 281 | P-value |
| Age | |||||||||
| <10 | 22 (85%) | 837 (92%) | 0.27 | 13 (93%) | 585 (93%) | 1.00 | 9 (75%) | 252 (90%) | 0.132 |
| ≥10 | 4 (15%) | 76 (8%) | 1 (7%) | 47 (7%) | 3 (25%) | 29 (10%) | |||
| Median (IQR) | 5.00 (3.03–6.21) | 4.00 (3.00–6.00) | 0.434 | 4.50 (3.19–5.96) | 4.00 (3.00–6.00) | 0.741 | 5.35 (2.67–8.75) | 4.00 (3.00–6.00) | 0.486 |
| Sex | 0.844 | 1.00 | 1.00 | ||||||
| Male | 13 (50%) | 478 (52%) | 7 (50%) | 335 (53%) | 6 (50%) | 143 (51%) | |||
| Female | 13 (50%) | 435 (48%) | 7 (50%) | 297 (47%) | 6 (50%) | 138 (49%) | |||
| WCC | |||||||||
| <50 × 109/L | 19 (73%) | 769 (84%) | 0.168 | 11 (79%) | 546 (86%) | 0.421 | 8 (67%) | 223 (79%) | 0.288 |
| ≥ 50 × 109/L | 7 (27%) | 143 (16%) | 3 (21%) | 85 (13%) | 4 (33%) | 58 (21%) | |||
| Median (IQR) | 16.35 (6.02–47.53) | 10.34 (5.20–30.30) | 0.268 | 16.35 (6.03–31.95) | 9.80 (5.02–25.65) | 0.572 | 16.00 (9.47–130.40) | 11.40 (5.60–40.80) | 0.34 |
| CNS disease | 1.00 | 1.00 | Unknown | Unknown | |||||
| Yes | 0 | 1 (<0.5%) | 0 (<0.5%) | 1 (<0.5%) | |||||
| No | 5 (19%) | 226 (25%) | 5 (36%) | 226 (36%) | |||||
| Clinical remission | 1.00 | 1.00 | 1.00 | ||||||
| Yes | 26 (100%) | 908 (99%) | 14 (100%) | 629 (100%) | 12 (100%) | 279 (99%) | |||
| No | 0 | 4 (<0.5%) | 0 | 2 (<0.5%) | 0 | 2 (1%) | |||
| Induction death | 0 | 1 (<0.5%) | 0 | 1 (<0.5%) | 0 | 0 | |||
| Outcome | |||||||||
| 5-y EFS | 79% (63%-97%) | 92% (90%-94%) | 0.021 | 100% | 93% (91%-95%) | 0.341 | 58% (36%-94%) | 91% (87%-94%) | <0.001 |
| 5-y RR | 18% (0%-33%) | 6% (4%-8%) | 0.026 | 0% | 5% (3%-7%) | 0.41 | 37% (0%-59%) | 7% (4%-10%) | <0.001 |
| 5-y OS | 87% (74%-100%) | 97% (96%-98%) | 0.013 | 100% | 98% (67%-99%) | 0.626 | 74% (53%-100%) | 95% (92%-98%) | 0.009 |
| Hazard ratio EFS | 2.80 (1.10–6.90) | Reference | 0.027 | No event | Reference | 5.10 (1.90–13.00) | Reference | <0.001 | |
| Follow-up in years, median (IQR) | 6.72 (4.65–8.71) | 7.40 (5.45–9.22%) | 6.28 (2.25–8.57) | 7.30 (5.71–9.13) | 7.28 (5.84–9.13) | 7.67 (5.08–9.73) | |||
Data are n (%), rates at 5 y (95% CI) or median (IQR).
WCC = white blood cell count; CNS = central nervous system; CI = confidence interval; EFS = event-free survival; IQR = inter-quartile range; OS = overall survival; RR = relapse rate.