Outcome measures in pediatric AEL
Variable . | n . | 5-y OS, % ± 2SE% . | 5-y EFS, % ± 2SE% . |
---|---|---|---|
Morphology | |||
M0-M5, M7 FAB | 1910 | 65 ± 2* | 50 ± 2* |
M6 FAB | 24 | 56 ± 21 | 41 ± 20 |
PEL | 5 | 20 ± 36† | 20 ± 36† |
Erythroid/myeloid leukemia | 19 | 66 ± 23* | 46 ± 23* |
MDS (new WHO classification) | 10 | 56 ± 33 | 48 ± 33 |
AML (new WHO classification) | 5 | 80 ± 36 | 40 ± 44 |
Multilineage dysplasia | |||
Absent | 16 | 48 ± 26 | 38 ± 24 |
Present | 8 | 73 ± 33 | 50 ± 35 |
Karyotype | |||
Not complex | 17 | 55 ± 26 | 34 ± 24 |
Complex | 7 | 57 ± 37 | 57 ± 37 |
Mutations | |||
WT1 wild type | 9 | 53 ± 35 | 56 ± 33 |
WT1 mutation | 5 | 60 ± 44 | 20 ± 36 |
TP53 wild type | 10 | 57 ± 23 | 40 ± 31 |
TP53 mutation | 1 | 0 ± 0 | 0 ± 0‡ |
Fusion status | |||
No fusions | 13 | 45 ± 28 | 38 ± 27 |
Fusion positive | 9 | 65 ± 33 | 30 ± 33 |
NUP98 fusion positive | 7 | 54 ± 40 | 43 ± 37 |
NUP98–KDM5A positive | 4 | 25 ± 43 | 25 ± 43 |
NUP98–NSD1 positive | 2 | 100 ± 0 | NA |
NUP98–SET positive | 1 | 100 ± 0 | 100 ± 0 |
NPM1–MLF1 positive | 1 | NA | 0 ± 0 |
RUNX1–MECOM positive | 1 | NA | 0 ± 0 |
Morphology + fusion | |||
PEL without NUP98 fusion | 2 | 0 ± 0 | 0 ± 0 |
PEL with NUP98 fusion | 3 | 33 ± 54 | 33 ± 54 |
PEL with NUP98–KDM5A fusion | 2 | 0 ± 0 | 0 ± 0 |
MDS with NUP98 fusion | 3 | 50 ± 71 | 67 ± 54 |
MDS with NUP98–KDM5A fusion | 2 | 50 ± 71 | 50 ± 71 |
AML (nonerythroid) with NUP98–NSD1 fusion | 1 | 100 ± 0 | 0 ± 0 |
Variable . | n . | 5-y OS, % ± 2SE% . | 5-y EFS, % ± 2SE% . |
---|---|---|---|
Morphology | |||
M0-M5, M7 FAB | 1910 | 65 ± 2* | 50 ± 2* |
M6 FAB | 24 | 56 ± 21 | 41 ± 20 |
PEL | 5 | 20 ± 36† | 20 ± 36† |
Erythroid/myeloid leukemia | 19 | 66 ± 23* | 46 ± 23* |
MDS (new WHO classification) | 10 | 56 ± 33 | 48 ± 33 |
AML (new WHO classification) | 5 | 80 ± 36 | 40 ± 44 |
Multilineage dysplasia | |||
Absent | 16 | 48 ± 26 | 38 ± 24 |
Present | 8 | 73 ± 33 | 50 ± 35 |
Karyotype | |||
Not complex | 17 | 55 ± 26 | 34 ± 24 |
Complex | 7 | 57 ± 37 | 57 ± 37 |
Mutations | |||
WT1 wild type | 9 | 53 ± 35 | 56 ± 33 |
WT1 mutation | 5 | 60 ± 44 | 20 ± 36 |
TP53 wild type | 10 | 57 ± 23 | 40 ± 31 |
TP53 mutation | 1 | 0 ± 0 | 0 ± 0‡ |
Fusion status | |||
No fusions | 13 | 45 ± 28 | 38 ± 27 |
Fusion positive | 9 | 65 ± 33 | 30 ± 33 |
NUP98 fusion positive | 7 | 54 ± 40 | 43 ± 37 |
NUP98–KDM5A positive | 4 | 25 ± 43 | 25 ± 43 |
NUP98–NSD1 positive | 2 | 100 ± 0 | NA |
NUP98–SET positive | 1 | 100 ± 0 | 100 ± 0 |
NPM1–MLF1 positive | 1 | NA | 0 ± 0 |
RUNX1–MECOM positive | 1 | NA | 0 ± 0 |
Morphology + fusion | |||
PEL without NUP98 fusion | 2 | 0 ± 0 | 0 ± 0 |
PEL with NUP98 fusion | 3 | 33 ± 54 | 33 ± 54 |
PEL with NUP98–KDM5A fusion | 2 | 0 ± 0 | 0 ± 0 |
MDS with NUP98 fusion | 3 | 50 ± 71 | 67 ± 54 |
MDS with NUP98–KDM5A fusion | 2 | 50 ± 71 | 50 ± 71 |
AML (nonerythroid) with NUP98–NSD1 fusion | 1 | 100 ± 0 | 0 ± 0 |