Characteristics of the 3 molecular groups of post-ET/PV AML
| Patient characteristics . | Short-term transformation (n = 7) . | Intermediate transformations (n = 32) . | Long-term transformation (n = 10) . | P . |
|---|---|---|---|---|
| Sex, male/female | 4/3 | 17/15 | 9/1 | 0.12 |
| Diagnosis, PV/ET | 4/3 | 16/16 | 4/6 | 0.84 |
| Age at diagnosis of chronic phase (range), y | 66 (60-69) | 65 (26-82) | 53 (26-68) | 0.05 |
| Driver mutation, n (%) | 0.08 | |||
| JAK2V617F | 7 (100) | 27 (84) | 5 (50) | |
| CALR | — | 2 (6) | 4 (40) | |
| MPL | — | 2 (6) | — | |
| Triple negative | 1 (4) | 1 (10) | ||
| Time to leukemic transformation (range), y | 3 (1-12) | 10 (2-20) | 21 (12-30) | NT |
| Age at diagnosis of leukemic transformation (range), y | 67 (55-79) | 74 (38-88) | 75 (54-86) | NT |
| Additional mutations at the AML stage | ||||
| Mutations, n (range) | 7 (5-8) | 3 (1-8) | 4 (1-5) | 0.001 |
| TP53, n (%) | 0 | 17 (53) | 5 (50) | 0.03 |
| TET2, n (%) | 3 (42) | 8 (25) | 0 | 0.10 |
| RUNX1, n (%) | 5 (71) | 4 (13) | 2 (20) | 0.004 |
| ASXL1, n (%) | 1 (14) | 7 (22) | 4 (40) | 0.49 |
| EZH2, n (%) | 2 (28) | 6 (19) | 0 | 0.22 |
| DNMT3A, n (%) | 3 (42) | 4 (13) | 1 (10) | 0.17 |
| NRAS, n (%) | 0 | 2 (9) | 4 (40) | 0.02 |
| U2AF1, n (%) | 2 (28) | 4 (13) | 0 | 0.2 |
| IDH1/2, n (%) | 4 (57) | 3 (9) | 0 | 0.01 |
| Karyotype at the AML stage, n (%) | n = 6 | n = 23 | n = 6 | 0.31 |
| Normal | 4 (66) | 7 (30) | 2 (33) | |
| Abnormal not complex | 1 (17) | 3 (13) | 2 (33) | |
| Complex | 1 (17) | 8 (35) | 0 | |
| Monosomal | 0 | 5 (22) | 2 (33) | |
| Post-AML treatment, n (%) | 0.09 | |||
| Best supportive care | 2 (29) | 17 (53) | 4 (40) | |
| Low-dose cytarabine | 0 | 2 (6) | 0 | |
| Azacytidine | 3 (42) | 6 (19) | 5 (50) | |
| Intensive chemotherapy | 2 (29) | 7 (22) | 1 (10) | |
| Allograft | 2 (29) | 3 (9) | 2 (20) | |
| Post-AML events | NT | |||
| Deaths, n (%) | 4 (57) | 26 (91) | 7 (70) | |
| Median survival, mo | 19 | 4 | 8 |
| Patient characteristics . | Short-term transformation (n = 7) . | Intermediate transformations (n = 32) . | Long-term transformation (n = 10) . | P . |
|---|---|---|---|---|
| Sex, male/female | 4/3 | 17/15 | 9/1 | 0.12 |
| Diagnosis, PV/ET | 4/3 | 16/16 | 4/6 | 0.84 |
| Age at diagnosis of chronic phase (range), y | 66 (60-69) | 65 (26-82) | 53 (26-68) | 0.05 |
| Driver mutation, n (%) | 0.08 | |||
| JAK2V617F | 7 (100) | 27 (84) | 5 (50) | |
| CALR | — | 2 (6) | 4 (40) | |
| MPL | — | 2 (6) | — | |
| Triple negative | 1 (4) | 1 (10) | ||
| Time to leukemic transformation (range), y | 3 (1-12) | 10 (2-20) | 21 (12-30) | NT |
| Age at diagnosis of leukemic transformation (range), y | 67 (55-79) | 74 (38-88) | 75 (54-86) | NT |
| Additional mutations at the AML stage | ||||
| Mutations, n (range) | 7 (5-8) | 3 (1-8) | 4 (1-5) | 0.001 |
| TP53, n (%) | 0 | 17 (53) | 5 (50) | 0.03 |
| TET2, n (%) | 3 (42) | 8 (25) | 0 | 0.10 |
| RUNX1, n (%) | 5 (71) | 4 (13) | 2 (20) | 0.004 |
| ASXL1, n (%) | 1 (14) | 7 (22) | 4 (40) | 0.49 |
| EZH2, n (%) | 2 (28) | 6 (19) | 0 | 0.22 |
| DNMT3A, n (%) | 3 (42) | 4 (13) | 1 (10) | 0.17 |
| NRAS, n (%) | 0 | 2 (9) | 4 (40) | 0.02 |
| U2AF1, n (%) | 2 (28) | 4 (13) | 0 | 0.2 |
| IDH1/2, n (%) | 4 (57) | 3 (9) | 0 | 0.01 |
| Karyotype at the AML stage, n (%) | n = 6 | n = 23 | n = 6 | 0.31 |
| Normal | 4 (66) | 7 (30) | 2 (33) | |
| Abnormal not complex | 1 (17) | 3 (13) | 2 (33) | |
| Complex | 1 (17) | 8 (35) | 0 | |
| Monosomal | 0 | 5 (22) | 2 (33) | |
| Post-AML treatment, n (%) | 0.09 | |||
| Best supportive care | 2 (29) | 17 (53) | 4 (40) | |
| Low-dose cytarabine | 0 | 2 (6) | 0 | |
| Azacytidine | 3 (42) | 6 (19) | 5 (50) | |
| Intensive chemotherapy | 2 (29) | 7 (22) | 1 (10) | |
| Allograft | 2 (29) | 3 (9) | 2 (20) | |
| Post-AML events | NT | |||
| Deaths, n (%) | 4 (57) | 26 (91) | 7 (70) | |
| Median survival, mo | 19 | 4 | 8 |
Quantitative variables are summarized as median and ranges.
sAML, secondary AML; NT, not tested.