Patients' characteristics
| Variable . | All patients (total n = 2169) . | SR, % (n = 758) . | MR, % (n = 1157) . | HR, % (n = 254) . | ||
|---|---|---|---|---|---|---|
| N* . | % . | 6y-pEFS, % (SE) . | ||||
| All | 2169 | 100 | 79.6 (0.9) | 100 | 100 | 100 |
| Sex | ||||||
| Male | 1226 | 56.5 | 78.4 (1.2) | 54.9 | 56.4 | 61.8 |
| Female | 943 | 43.5 | 81.1 (1.3) | 45.1 | 43.6 | 38.2 |
| Age† | ||||||
| Less than 1 y | 34 | 1.6 | 40.4 (8.5) | 0 | 1.5 | 6.7 |
| 1 to less than 6 y | 1255 | 57.9 | 84.3 (1.0) | 100 | 35.1 | 35.8 |
| 6 to less than 10 y | 447 | 20.6 | 80.3 (1.9) | 0 | 33.1 | 25.2 |
| 10 to less than 15 y | 340 | 15.7 | 70.5 (2.5) | 0 | 23.9 | 25.2 |
| 15 y and older | 93 | 4.3 | 58.3 (5.4) | 0 | 6.5 | 7.1 |
| Initial WBC (/μ L) | ||||||
| Less than 10 000 | 1071 | 49.4 | 84.2 (1.1) | 76.8 | 38.8 | 15.7 |
| 10 000 to less than 20 × 109/L | 319 | 14.7 | 83.9 (2.1) | 23.2 | 9.8 | 11.8 |
| 20 000 to less than 50 × 109/L | 362 | 16.7 | 79.4 (2.2) | 0 | 27.5 | 17.3 |
| 50 000 to less than 100 × 109/L | 180 | 8.3 | 74.0 (3.3) | 0 | 12.2 | 15.4 |
| 100 000 to less than 200 × 109/L | 111 | 5.1 | 62.9 (4.6) | 0 | 6.5 | 14.2 |
| 200 000 and over | 126 | 5.8 | 52.6 (4.5) | 0 | 5.3 | 25.6 |
| BFM-RF | ||||||
| Less than 0.8 | 765 | 35.8 | 86.2 (1.3) | 53.6 | 30.1 | 8.7 |
| More than 0.8 | 1372 | 64.2 | 75.8 (1.2) | 46.4 | 69.9 | 91.3 |
| CNS status | ||||||
| CNS1 | 1717 | 79.5 | 81.3 (1.0) | 84.7 | 77.9 | 71.0 |
| CNS2 | 112 | 5.2 | 79.7 (3.9) | 3.3 | 5.9 | 7.5 |
| CNS3 | 64 | 3.0 | 57.7 (6.2) | 1.2 | 2.9 | 8.7 |
| TLP+ | 148 | 6.8 | 69.2 (3.8) | 4.1 | 8.2 | 9.1 |
| TLP− | 119 | 5.5 | 81.0 (3.7) | 6.7 | 5.1 | 3.6 |
| Immunophenotype | ||||||
| Precursor B | 1798 | 86.5 | 80.2 (1.0) | 100 | 82.6 | 65.5 |
| T | 277 | 13.3 | 74.8 (2.6) | 0 | 17.1 | 34.5 |
| Other‡ | 3 | 0.1 | 0 | 0.3 | 0 | |
| DNA index | ||||||
| Less than 1.16 | 1187 | 78.7 | 76.2 (1.3) | 65.4 | 83.2 | 91.9 |
| More than 1.16 | 322 | 21.3 | 88.9 (1.8) | 34.6 | 16.8 | 8.1 |
| TEL/AML1 | ||||||
| Negative | 916 | 78.6 | 75.1 (1.4) | 73.2 | 78.7 | 94.4 |
| Positive | 250 | 21.4 | 91.2 (1.8) | 26.8 | 21.3 | 5.6 |
| BCR/ABL | ||||||
| Negative | 1918 | 97.9 | 80.4 (0.9) | 100 | 100 | 82.4 |
| Positive | 42 | 2.1 | 26.2 (6.8) | 0 | 0 | 17.6 |
| MLL/AF4 | ||||||
| Negative | 1154 | 97.9 | 77.4 (1.2) | 100 | 100 | 86.9 |
| Positive | 25 | 2.1 | 40.0 (9.8) | 0 | 0 | 13.1 |
| Non-T lineage NCI risk criteria‖ | ||||||
| Standard risk | 1256 | 71.0 | 86.5 (1.0) | 100 | 54.1 | 34.9 |
| High risk | 512 | 29.0 | 67.4 (2.1) | 0 | 45.9 | 65.1 |
| T lineage NCI risk criteria‖ | ||||||
| Standard risk | 72 | 26.2 | 90.1 (3.5) | 33.3 | 10.5 | |
| High risk | 203 | 73.8 | 69.2 (3.3) | 66.7 | 89.5 | |
| Prednisone response | ||||||
| Good | 1963 | 91.4 | 82.1 (0.9) | 100 | 100 | 27.2 |
| Poor | 184 | 8.6 | 55.0 (3.7) | 0 | 0 | 72.7 |
| BM day 15 | ||||||
| M1 | 880 | 61.5 | 87.1 (1.1) | 72.2 | 65.0 | 22.3 |
| M2 | 365 | 25.5 | 75.5 (2.3) | 24.1 | 25.3 | 29.9 |
| M3 | 186 | 13.0 | 47.3 (3.7) | 3.8 | 9.7 | 47.7 |
| Nonremission day 33 | ||||||
| No | 2120 | 97.7 | 80.6 (0.9) | 100 | 100 | 80.7 |
| Yes | 49 | 2.3 | 36.3 (6.9) | 0 | 0 | 19.3 |
| Variable . | All patients (total n = 2169) . | SR, % (n = 758) . | MR, % (n = 1157) . | HR, % (n = 254) . | ||
|---|---|---|---|---|---|---|
| N* . | % . | 6y-pEFS, % (SE) . | ||||
| All | 2169 | 100 | 79.6 (0.9) | 100 | 100 | 100 |
| Sex | ||||||
| Male | 1226 | 56.5 | 78.4 (1.2) | 54.9 | 56.4 | 61.8 |
| Female | 943 | 43.5 | 81.1 (1.3) | 45.1 | 43.6 | 38.2 |
| Age† | ||||||
| Less than 1 y | 34 | 1.6 | 40.4 (8.5) | 0 | 1.5 | 6.7 |
| 1 to less than 6 y | 1255 | 57.9 | 84.3 (1.0) | 100 | 35.1 | 35.8 |
| 6 to less than 10 y | 447 | 20.6 | 80.3 (1.9) | 0 | 33.1 | 25.2 |
| 10 to less than 15 y | 340 | 15.7 | 70.5 (2.5) | 0 | 23.9 | 25.2 |
| 15 y and older | 93 | 4.3 | 58.3 (5.4) | 0 | 6.5 | 7.1 |
| Initial WBC (/μ L) | ||||||
| Less than 10 000 | 1071 | 49.4 | 84.2 (1.1) | 76.8 | 38.8 | 15.7 |
| 10 000 to less than 20 × 109/L | 319 | 14.7 | 83.9 (2.1) | 23.2 | 9.8 | 11.8 |
| 20 000 to less than 50 × 109/L | 362 | 16.7 | 79.4 (2.2) | 0 | 27.5 | 17.3 |
| 50 000 to less than 100 × 109/L | 180 | 8.3 | 74.0 (3.3) | 0 | 12.2 | 15.4 |
| 100 000 to less than 200 × 109/L | 111 | 5.1 | 62.9 (4.6) | 0 | 6.5 | 14.2 |
| 200 000 and over | 126 | 5.8 | 52.6 (4.5) | 0 | 5.3 | 25.6 |
| BFM-RF | ||||||
| Less than 0.8 | 765 | 35.8 | 86.2 (1.3) | 53.6 | 30.1 | 8.7 |
| More than 0.8 | 1372 | 64.2 | 75.8 (1.2) | 46.4 | 69.9 | 91.3 |
| CNS status | ||||||
| CNS1 | 1717 | 79.5 | 81.3 (1.0) | 84.7 | 77.9 | 71.0 |
| CNS2 | 112 | 5.2 | 79.7 (3.9) | 3.3 | 5.9 | 7.5 |
| CNS3 | 64 | 3.0 | 57.7 (6.2) | 1.2 | 2.9 | 8.7 |
| TLP+ | 148 | 6.8 | 69.2 (3.8) | 4.1 | 8.2 | 9.1 |
| TLP− | 119 | 5.5 | 81.0 (3.7) | 6.7 | 5.1 | 3.6 |
| Immunophenotype | ||||||
| Precursor B | 1798 | 86.5 | 80.2 (1.0) | 100 | 82.6 | 65.5 |
| T | 277 | 13.3 | 74.8 (2.6) | 0 | 17.1 | 34.5 |
| Other‡ | 3 | 0.1 | 0 | 0.3 | 0 | |
| DNA index | ||||||
| Less than 1.16 | 1187 | 78.7 | 76.2 (1.3) | 65.4 | 83.2 | 91.9 |
| More than 1.16 | 322 | 21.3 | 88.9 (1.8) | 34.6 | 16.8 | 8.1 |
| TEL/AML1 | ||||||
| Negative | 916 | 78.6 | 75.1 (1.4) | 73.2 | 78.7 | 94.4 |
| Positive | 250 | 21.4 | 91.2 (1.8) | 26.8 | 21.3 | 5.6 |
| BCR/ABL | ||||||
| Negative | 1918 | 97.9 | 80.4 (0.9) | 100 | 100 | 82.4 |
| Positive | 42 | 2.1 | 26.2 (6.8) | 0 | 0 | 17.6 |
| MLL/AF4 | ||||||
| Negative | 1154 | 97.9 | 77.4 (1.2) | 100 | 100 | 86.9 |
| Positive | 25 | 2.1 | 40.0 (9.8) | 0 | 0 | 13.1 |
| Non-T lineage NCI risk criteria‖ | ||||||
| Standard risk | 1256 | 71.0 | 86.5 (1.0) | 100 | 54.1 | 34.9 |
| High risk | 512 | 29.0 | 67.4 (2.1) | 0 | 45.9 | 65.1 |
| T lineage NCI risk criteria‖ | ||||||
| Standard risk | 72 | 26.2 | 90.1 (3.5) | 33.3 | 10.5 | |
| High risk | 203 | 73.8 | 69.2 (3.3) | 66.7 | 89.5 | |
| Prednisone response | ||||||
| Good | 1963 | 91.4 | 82.1 (0.9) | 100 | 100 | 27.2 |
| Poor | 184 | 8.6 | 55.0 (3.7) | 0 | 0 | 72.7 |
| BM day 15 | ||||||
| M1 | 880 | 61.5 | 87.1 (1.1) | 72.2 | 65.0 | 22.3 |
| M2 | 365 | 25.5 | 75.5 (2.3) | 24.1 | 25.3 | 29.9 |
| M3 | 186 | 13.0 | 47.3 (3.7) | 3.8 | 9.7 | 47.7 |
| Nonremission day 33 | ||||||
| No | 2120 | 97.7 | 80.6 (0.9) | 100 | 100 | 80.7 |
| Yes | 49 | 2.3 | 36.3 (6.9) | 0 | 0 | 19.3 |
CR indicates complete remission; BM, bone marrow; CNS, central nervous system; SR, standard risk; MR, medium risk; HR, high risk.
Data refer to patients with successful investigation of the respective criteria.
Median age was 5.0 years (range, 0.07-17.92 years). Nineteen additional patients younger than 1 year were treated in the Interfant-99 pilot study43 and were not included in the analyses.
Two patients had the immunophenotype of a mature B-cell leukemia (cytomorphologically FAB L1); one patient had a biphenotypic acute leukemia.
NCI-SR, age 1 or younger and less than 10 years, and WBC less than 50 × 109/L; NCI-HR, age 10 years or older or WBC 50 × 109/L or more. Infants less than 1 year are excluded from the NCI definition.
One patient of the SR group was falsely BCR/ABL-negative at initial diagnosis (BCR/ABL-positive in relapse and in subsequently repeated analysis of the initial material).