Clinical characteristics of pediatric cases with t(16;21)
| . | FUS-ERG . | RUNX1-CBFA2T3 . | I-BFM reference cohort . |
|---|---|---|---|
| N | 31 | 23 | 1326 |
| Median age (range), y | 8.5 (2.0-17.5) | 6.8 (1-17) | 8.7 (0-20.3) |
| Sex | |||
| % male | 61 | 46 | 51.6 |
| Median WBC (range), ×109/L | 14.0 (1-203) | 12.5 (0.01-185)* | 19.5 (0.01-190) |
| <20, n (%) | 17 (54.8) | 13 (61.9) | 670 (50.5) |
| 20-100, n (%) | 10 (32.3) | 7 (33.3) | 414 (31.2) |
| >100, n (%) | 5 (12.9) | 1 (4.8) | 242 (18.3) |
| FAB-type, n (%) | |||
| M0 | 3 (9.6) | 1 (4.3) | 46 (3.7) |
| M1 | 8 (25.8) | 3 (13.0) | 178 (14.5) |
| M2 | 8 (25.8) | 10 (43.5)* | 339 (27.6) |
| M4 | 6 (19.3) | 2 (8.7) | 293 (23.9) |
| M5 | 4 (12.9) | 1 (4.3) | 258 (21.6) |
| M6 | — | — | 29 (2.4) |
| M7 | 1 (3.2) | 1 (4.3) | 85 (6.9) |
| NOS | 1 (3.2) | 5 (21.7) | — |
| CNS involvement, n (%) | 6 (18.1) | 5 (22.7) | |
| Cytogenetics, n (%) | |||
| Sole abnormality | 12 (36.4) | 4 (21.1) | |
| Trisomy 8 | 6 (18.1) | 7 (36.8) | |
| Trisomy 10 | 4 (12.1) | — | |
| Complex karyotype | 10 (30.3) | 5 (26.3) | |
| Treatment, n (%) | |||
| CR obtained | 28 (87.5) | 22 (95.6) | |
| Refractory disease | 3 (9.4) | — | |
| HSCT in CR1 | 13 (40.6) | 8 (34.8) | |
| Survival (SE), % | |||
| 4-y EFS | 13 (9)† | 77 (9)† | 51 (1) |
| 4-y OS | 26 (8)† | 81 (8)† | 68 (1) |
| 4-y CIR | 69 (8)† | 0 (0)† | 32 (1) |
| . | FUS-ERG . | RUNX1-CBFA2T3 . | I-BFM reference cohort . |
|---|---|---|---|
| N | 31 | 23 | 1326 |
| Median age (range), y | 8.5 (2.0-17.5) | 6.8 (1-17) | 8.7 (0-20.3) |
| Sex | |||
| % male | 61 | 46 | 51.6 |
| Median WBC (range), ×109/L | 14.0 (1-203) | 12.5 (0.01-185)* | 19.5 (0.01-190) |
| <20, n (%) | 17 (54.8) | 13 (61.9) | 670 (50.5) |
| 20-100, n (%) | 10 (32.3) | 7 (33.3) | 414 (31.2) |
| >100, n (%) | 5 (12.9) | 1 (4.8) | 242 (18.3) |
| FAB-type, n (%) | |||
| M0 | 3 (9.6) | 1 (4.3) | 46 (3.7) |
| M1 | 8 (25.8) | 3 (13.0) | 178 (14.5) |
| M2 | 8 (25.8) | 10 (43.5)* | 339 (27.6) |
| M4 | 6 (19.3) | 2 (8.7) | 293 (23.9) |
| M5 | 4 (12.9) | 1 (4.3) | 258 (21.6) |
| M6 | — | — | 29 (2.4) |
| M7 | 1 (3.2) | 1 (4.3) | 85 (6.9) |
| NOS | 1 (3.2) | 5 (21.7) | — |
| CNS involvement, n (%) | 6 (18.1) | 5 (22.7) | |
| Cytogenetics, n (%) | |||
| Sole abnormality | 12 (36.4) | 4 (21.1) | |
| Trisomy 8 | 6 (18.1) | 7 (36.8) | |
| Trisomy 10 | 4 (12.1) | — | |
| Complex karyotype | 10 (30.3) | 5 (26.3) | |
| Treatment, n (%) | |||
| CR obtained | 28 (87.5) | 22 (95.6) | |
| Refractory disease | 3 (9.4) | — | |
| HSCT in CR1 | 13 (40.6) | 8 (34.8) | |
| Survival (SE), % | |||
| 4-y EFS | 13 (9)† | 77 (9)† | 51 (1) |
| 4-y OS | 26 (8)† | 81 (8)† | 68 (1) |
| 4-y CIR | 69 (8)† | 0 (0)† | 32 (1) |