Table 3.
Features and Treatment Outcome of High Hyperdiploid Acute Lymphoblastic Leukemia
| High Hyperdiploid | All Protocols | MRD-guided Protocols Only | Non-MRD-guided Protocols Only | ||||||
|---|---|---|---|---|---|---|---|---|---|
| IKZF1 | IKZF1 | IKZF1 | |||||||
| Deleted | Wild-type | Deleted | Wild-type | Deleted | Wild-type | ||||
| n = 968 | n = 85 | n = 883 | P-value | n= 65 | n = 631 | P-value | n = 20 | n = 252 | P-value |
| Age | |||||||||
| <10 | 68 (80%) | 780 (88%) | 0.037 | 51 (78%) | 556 (88%) | 0.032 | 17 (85%) | 224 (89%) | 0.485 |
| ≥10 | 17 (20%) | 103 (12%) | 14 (22%) | 75 (12%) | 3 (15%) | 28 (11%) | |||
| Median (IQR) | 5.00 (3.09–7.83) | 4.00 (2.52–6.45) | 0.005 | 5.36 (3.28–7.92) | 3.94 (2.60–6.41) | 0.003 | 4.00 (2.91–6.55) | 4.00 (2.00–6.86) | 0.676 |
| Sex | 0.734 | 1.00 | 0.493 | ||||||
| Male | 42 (49%) | 457 (52%) | 33 (51%) | 322 (51%) | 9 (45%) | 135 (54%) | |||
| Female | 43 (51%) | 426 (48%) | 32 (49%) | 309 (49%) | 11 (55%) | 117 (46%) | |||
| WCC | |||||||||
| <50 × 109/L | 76 (89%) | 803 (91%) | 0.557 | 60 (92%) | 577 (91%) | 1.00 | 16 (80%) | 226 (90%) | 0.247 |
| ≥50 × 109/L | 9 (11%) | 79 (9%) | 5 (8%) | 54 (9%) | 4 (20%) | 25 (10%) | |||
| Median (IQR) | 7.20 (3.40–17.80) | 7.30 (3.50–18.35) | 0.922 | 7.10 (3.40–16.50) | 7.00 (3.40–17.30) | 0.781 | 15.95 (3.32–32.59) | 8.10 (3.89–21.80) | 0.599 |
| CNS disease | 1.00 | 1.00 | |||||||
| Yes | 0 | 2 (<0.5%) | 0 | 2 (0.3%) | Unknown | Unknown | |||
| No | 17 (20%) | 204 (23%) | 17 (26%) | 204 (32%) | |||||
| Clinical remission | 1.00 | 1.00 | |||||||
| Yes | 84 (99%) | 870 (99%) | 65 (100%) | 623 (99%) | 19 (95%) | 247 (98%) | 0.37 | ||
| No | 1 (1%) | 8 (1%) | 0 | 5 (1%) | 1 (5%) | 3 (1%) | |||
| Induction death | 0 | 5 (1%) | 0 | 3 (<0.5%) | 0 | 2 (1%) | |||
| Outcome | |||||||||
| 5-y EFS | 76% (67%-87%) | 89% (87%-91%) | 0.006 | 73% (63%-86%) | 88% (86%-91%) | 0.004 | 85% (70%-100%) | 90% (87-94%) | 0.62 |
| 5-y RR | 20% (10%-28%) | 8% (6%-10%) | 0.003 | 23% (10%-33%) | 9% (6%-11%) | 0.002 | 11% (0%-24%) | 6% (3-9%) | 0.576 |
| 5-y OS | 88% (81%-96%) | 94% (93%-96%) | 0.005 | 88% (79%-97%) | 94% (92%-96%) | 0.004 | 90% (78%-100%) | 94% (91-97%) | 0.541 |
| Hazard ratio EFS | 2.00 (1.20–3.30) | Reference | 0.007 | 2.20 (1.30%–3.90) | Reference | 0.005 | 1.40 (0.41%–4.50) | Reference | 0.621 |
| Follow-up in years, median (IQR) | 6.93 (4.48–9.83) | 7.19 (4.89–9.10) | 6.90 (4.48–8.96) | 6.95 (4.70–8.86) | 8.64 (4.68%–10.52) | 7.62 (5.68–10.43) | |||
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.