Baseline characteristics stratified by MPN driver mutations
| . | Study patients (N = 122) . | JAK2 mutated (n = 67) . | CALR mutated (n = 16) . | MPL mutated (n = 7) . | Triple negative (n = 32) . | P, JAK2/MPL vs CALR vs triple negative . |
|---|---|---|---|---|---|---|
| Male, n (%) | 72 (59) | 41 (61) | 10 (63) | 6 (86) | 15 (47) | .27 |
| MPN diagnosis at AP/BP, n (%) | <.0001 | |||||
| PV/PPV-MF | 40 (33) | 36 (54) | 0 (0) | 0 (0) | 4 (13) | |
| ET/PET-MF | 38 (31) | 16 (24) | 11 (69) | 2 (29) | 9 (28) | |
| PMF | 34 (28) | 12 (18) | 5 (31) | 5 (71) | 12 (38) | |
| MPN-U | 10 (8) | 3 (5) | 0 (0) | 0 (0) | 7 (22) | |
| Age at AP/BP, median (range), y | 69 (36-86) | 69 (45-84) | 67 (47-79) | 70 (47-86) | 71 (36-86) | .08 |
| Time from MPN diagnosis to AP/BP, median (range), y | 5.4 (0.0-43.0) | 6.7 (0.1-43.0) | 10.9 (2.2-23.6) | 2.3 (0.3-6.5) | 2.8 (0.0-18.5) | .003 |
| Prior MPN therapies, n (%) | .84 | |||||
| 0 or 1 | 73 (60) | 37 (55) | 10 (63) | 5 (71) | 21 (66) | |
| 2 | 25 (21) | 13 (19) | 4 (25) | 2 (29) | 6 (19) | |
| ≥3 | 24 (20) | 17 (25) | 2 (13) | 0 (0) | 5 (16) | |
| Status at time of NGS, n (%) | .40 | |||||
| AP | 14 (11) | 6 (9) | 4 (25) | 1 (14) | 3 (9) | |
| AML* | 107 (88) | 60 (90) | 12 (75) | 6 (86) | 29 (91) | |
| MPAL | 1 (1) | 1 (2) | 0 (0) | 0 (0) | 0 (0) | |
| ECOG at AP/BP, n (%) | .51 | |||||
| 0 or 1 | 88 (72) | 51 (76) | 13 (81) | 5 (71) | 19 (59) | |
| 2 | 17 (14) | 9 (13) | 1 (6) | 0 (0) | 7 (22) | |
| ≥3 | 14 (12) | 7 (10) | 1 (6) | 2 (29) | 4 (13) | |
| Not available | 3 (3) | 0 (0) | 1 (6) | 0 (0) | 2 (6) | |
| Hemoglobin, median (range) (n = 120), g/L | 88 (51-183) | 91 (55-183) | 90 (52-124) | 89 (78-109) | 83 (51-111) | .05 |
| White blood cell count, median (range) (n = 121), ×109/L | 12.8 (0.8-278.0) | 12.0 (0.9-278.0) | 19.4 (1.3-138.8) | 18.7 (10.1-88.2) | 14.9 (0.8-150.0) | .94 |
| Platelets, median (range) (n = 120), ×109/L | 62 (7-1918) | 59 (8-1918) | 77 (9-865) | 112 (32-620) | 43 (7-863) | .13 |
| Blasts in PB, median (range) (n = 121), % | 24 (0-96) | 26 (0-96) | 24 (0-90) | 17 (7-60) | 24 (0-90) | .94 |
| Blasts in BM, median (range) (n = 100), % | 35 (0-95) | 40 (0-95) | 25 (3-90) | 20 (1-55) | 31 (0-90) | .73 |
| LDH, median (range), U/L | 624 (144-4274) | 618 (156-4000) | 1289 (354-4274) | 1548 (431-3410) | 465 (144-2168) | .01 |
| Albumin, median (range), g/L | 37 (23-48) | 37 (29-48) | 36 (27-43) | 37 (33-44) | 35 (23-46) | .09 |
| Spleen status, n (%) | .20 | |||||
| Splenectomy | 8 (7) | 4 (6) | 1 (6) | 1 (14) | 2 (6) | |
| Not palpable | 40 (33) | 19 (28) | 5 (31) | 1 (14) | 15 (47) | |
| Spleen ≤10 cm BCM | 30 (25) | 18 (27) | 3 (19) | 1 (14) | 8 (25) | |
| Spleen ≥11 cm BCM | 37 (30) | 23 (34) | 7 (23) | 3 (43) | 4 (13) | |
| Missing | 7 (6) | 3 (4) | 0 (0) | 1 (14) | 3 (9) | |
| Cytogenetic abnormalities, n (%) | .78 | |||||
| Normal | 30 (25) | 14 (28) | 6 (43) | 2 (50) | 8 (32) | |
| 1 | 18 (15) | 9 (18) | 3 (21) | 1 (25) | 5 (20) | |
| 2 | 14 (12) | 11 (22) | 0 (0) | 0 (0) | 3 (12) | |
| ≥3 | 32 (26) | 17 (33) | 5 (36) | 1 (25) | 9 (36) | |
| Failed | 11 (9) | 4 (6) | 2 (13) | 1 (14) | 4 (13) | |
| Not available | 17 (14) | 12 (18) | 0 (0) | 2 (29) | 3 (9) | |
| Co-occurrence of mutations, n (%) | ||||||
| ASXL1 | 36 (30) | 15 (22) | 7 (44) | 3 (43) | 11 (34) | .24 |
| TET2 | 30 (25) | 18 (49) | 3 (19) | 1 (14) | 8 (25) | .84 |
| SRSF2 | 27 (22) | 14 (21) | 1 (6) | 3 (43) | 9 (28) | .22 |
| RUNX1 | 25 (20) | 17 (25) | 3 (19) | 1 (14) | 4 (13) | .38 |
| TP53 | 21 (17) | 10 (15) | 4 (25) | 0 (0) | 7 (22) | .39 |
| DNMT3A | 20 (16) | 14 (21) | 4 (25) | 0 (0) | 2 (6) | .16 |
| IDH1 | 16 (13) | 9 (13) | 2 (13) | 1 (14) | 4 (13) | 1.00 |
| IDH2 | 16 (13) | 12 (18) | 1 (6) | 1 (14) | 2 (6) | .27 |
| STAG2 | 14 (11) | 10 (15) | 0 (0) | 0 (0) | 4 (13) | .30 |
| NRAS | 13 (11) | 8 (12) | 1 (6) | 0 (0) | 4 (13) | .84 |
| SETBP1 | 11 (9) | 1 (1) | 1 (6) | 1 (14) | 8 (25) | .002 |
| U2AF1 | 11 (9) | 6 (9) | 1 (6) | 1 (14) | 3 (9) | 1.00 |
| EZH2 | 9 (7) | 3 (4) | 4 (25) | 1 (14) | 1 (3) | .04 |
| SF3B1 | 9 (7) | 5 (7) | 0 (0) | 1 (14) | 3 (9) | .62 |
| KRAS | 8 (7) | 5 (7) | 0 (0) | 1 (14) | 2 (6) | .77 |
| PHF6 | 8 (7) | 6 (9) | 0 (0) | 0 (0) | 2 (6) | .77 |
| Number of mutations, n (%) | .09 | |||||
| 0-1 | 20 (16) | 7 (10) | 3 (19) | 0 (0) | 10 (31) | |
| 2-3 | 46 (38) | 28 (42) | 6 (38) | 3 (43) | 9 (28) | |
| ≥4 | 56 (46) | 32 (48) | 7 (44) | 4 (57) | 13 (41) | |
| Treatment received, n (%) | .15 | |||||
| Intensive | 43 (35) | 21 (31) | 8 (50) | 1 (14) | 13 (41) | |
| 3+7 | 20 (16) | 6 (9) | 3 (19) | 1 (14) | 10 (31) | |
| FLAG-IDA | 13 (11) | 9 (13) | 2 (13) | 0 (0) | 2 (6) | |
| NOVE-HIDAC | 10 (8) | 6 (9) | 3 (19) | 0 (0) | 1 (3) | |
| Transplant upfront | 1 (1) | 0 (0) | 0 (0) | 1 (14) | 0 (0) | |
| Nonintensive† | 27 (22) | 13 (19) | 5 (31) | 2 (29) | 7 (22) | |
| BSC | 51 (42) | 33 (49) | 3 (19) | 3 (43) | 12 (38) | |
| Allogeneic transplant, n (%) | .48 | |||||
| Yes | 19 (16) | 9 (14) | 4 (27) | 1 (17) | 5 (16) | |
| No | 103 (84) | 56 (86) | 11 (73) | 5 (83) | 27 (84) |
| . | Study patients (N = 122) . | JAK2 mutated (n = 67) . | CALR mutated (n = 16) . | MPL mutated (n = 7) . | Triple negative (n = 32) . | P, JAK2/MPL vs CALR vs triple negative . |
|---|---|---|---|---|---|---|
| Male, n (%) | 72 (59) | 41 (61) | 10 (63) | 6 (86) | 15 (47) | .27 |
| MPN diagnosis at AP/BP, n (%) | <.0001 | |||||
| PV/PPV-MF | 40 (33) | 36 (54) | 0 (0) | 0 (0) | 4 (13) | |
| ET/PET-MF | 38 (31) | 16 (24) | 11 (69) | 2 (29) | 9 (28) | |
| PMF | 34 (28) | 12 (18) | 5 (31) | 5 (71) | 12 (38) | |
| MPN-U | 10 (8) | 3 (5) | 0 (0) | 0 (0) | 7 (22) | |
| Age at AP/BP, median (range), y | 69 (36-86) | 69 (45-84) | 67 (47-79) | 70 (47-86) | 71 (36-86) | .08 |
| Time from MPN diagnosis to AP/BP, median (range), y | 5.4 (0.0-43.0) | 6.7 (0.1-43.0) | 10.9 (2.2-23.6) | 2.3 (0.3-6.5) | 2.8 (0.0-18.5) | .003 |
| Prior MPN therapies, n (%) | .84 | |||||
| 0 or 1 | 73 (60) | 37 (55) | 10 (63) | 5 (71) | 21 (66) | |
| 2 | 25 (21) | 13 (19) | 4 (25) | 2 (29) | 6 (19) | |
| ≥3 | 24 (20) | 17 (25) | 2 (13) | 0 (0) | 5 (16) | |
| Status at time of NGS, n (%) | .40 | |||||
| AP | 14 (11) | 6 (9) | 4 (25) | 1 (14) | 3 (9) | |
| AML* | 107 (88) | 60 (90) | 12 (75) | 6 (86) | 29 (91) | |
| MPAL | 1 (1) | 1 (2) | 0 (0) | 0 (0) | 0 (0) | |
| ECOG at AP/BP, n (%) | .51 | |||||
| 0 or 1 | 88 (72) | 51 (76) | 13 (81) | 5 (71) | 19 (59) | |
| 2 | 17 (14) | 9 (13) | 1 (6) | 0 (0) | 7 (22) | |
| ≥3 | 14 (12) | 7 (10) | 1 (6) | 2 (29) | 4 (13) | |
| Not available | 3 (3) | 0 (0) | 1 (6) | 0 (0) | 2 (6) | |
| Hemoglobin, median (range) (n = 120), g/L | 88 (51-183) | 91 (55-183) | 90 (52-124) | 89 (78-109) | 83 (51-111) | .05 |
| White blood cell count, median (range) (n = 121), ×109/L | 12.8 (0.8-278.0) | 12.0 (0.9-278.0) | 19.4 (1.3-138.8) | 18.7 (10.1-88.2) | 14.9 (0.8-150.0) | .94 |
| Platelets, median (range) (n = 120), ×109/L | 62 (7-1918) | 59 (8-1918) | 77 (9-865) | 112 (32-620) | 43 (7-863) | .13 |
| Blasts in PB, median (range) (n = 121), % | 24 (0-96) | 26 (0-96) | 24 (0-90) | 17 (7-60) | 24 (0-90) | .94 |
| Blasts in BM, median (range) (n = 100), % | 35 (0-95) | 40 (0-95) | 25 (3-90) | 20 (1-55) | 31 (0-90) | .73 |
| LDH, median (range), U/L | 624 (144-4274) | 618 (156-4000) | 1289 (354-4274) | 1548 (431-3410) | 465 (144-2168) | .01 |
| Albumin, median (range), g/L | 37 (23-48) | 37 (29-48) | 36 (27-43) | 37 (33-44) | 35 (23-46) | .09 |
| Spleen status, n (%) | .20 | |||||
| Splenectomy | 8 (7) | 4 (6) | 1 (6) | 1 (14) | 2 (6) | |
| Not palpable | 40 (33) | 19 (28) | 5 (31) | 1 (14) | 15 (47) | |
| Spleen ≤10 cm BCM | 30 (25) | 18 (27) | 3 (19) | 1 (14) | 8 (25) | |
| Spleen ≥11 cm BCM | 37 (30) | 23 (34) | 7 (23) | 3 (43) | 4 (13) | |
| Missing | 7 (6) | 3 (4) | 0 (0) | 1 (14) | 3 (9) | |
| Cytogenetic abnormalities, n (%) | .78 | |||||
| Normal | 30 (25) | 14 (28) | 6 (43) | 2 (50) | 8 (32) | |
| 1 | 18 (15) | 9 (18) | 3 (21) | 1 (25) | 5 (20) | |
| 2 | 14 (12) | 11 (22) | 0 (0) | 0 (0) | 3 (12) | |
| ≥3 | 32 (26) | 17 (33) | 5 (36) | 1 (25) | 9 (36) | |
| Failed | 11 (9) | 4 (6) | 2 (13) | 1 (14) | 4 (13) | |
| Not available | 17 (14) | 12 (18) | 0 (0) | 2 (29) | 3 (9) | |
| Co-occurrence of mutations, n (%) | ||||||
| ASXL1 | 36 (30) | 15 (22) | 7 (44) | 3 (43) | 11 (34) | .24 |
| TET2 | 30 (25) | 18 (49) | 3 (19) | 1 (14) | 8 (25) | .84 |
| SRSF2 | 27 (22) | 14 (21) | 1 (6) | 3 (43) | 9 (28) | .22 |
| RUNX1 | 25 (20) | 17 (25) | 3 (19) | 1 (14) | 4 (13) | .38 |
| TP53 | 21 (17) | 10 (15) | 4 (25) | 0 (0) | 7 (22) | .39 |
| DNMT3A | 20 (16) | 14 (21) | 4 (25) | 0 (0) | 2 (6) | .16 |
| IDH1 | 16 (13) | 9 (13) | 2 (13) | 1 (14) | 4 (13) | 1.00 |
| IDH2 | 16 (13) | 12 (18) | 1 (6) | 1 (14) | 2 (6) | .27 |
| STAG2 | 14 (11) | 10 (15) | 0 (0) | 0 (0) | 4 (13) | .30 |
| NRAS | 13 (11) | 8 (12) | 1 (6) | 0 (0) | 4 (13) | .84 |
| SETBP1 | 11 (9) | 1 (1) | 1 (6) | 1 (14) | 8 (25) | .002 |
| U2AF1 | 11 (9) | 6 (9) | 1 (6) | 1 (14) | 3 (9) | 1.00 |
| EZH2 | 9 (7) | 3 (4) | 4 (25) | 1 (14) | 1 (3) | .04 |
| SF3B1 | 9 (7) | 5 (7) | 0 (0) | 1 (14) | 3 (9) | .62 |
| KRAS | 8 (7) | 5 (7) | 0 (0) | 1 (14) | 2 (6) | .77 |
| PHF6 | 8 (7) | 6 (9) | 0 (0) | 0 (0) | 2 (6) | .77 |
| Number of mutations, n (%) | .09 | |||||
| 0-1 | 20 (16) | 7 (10) | 3 (19) | 0 (0) | 10 (31) | |
| 2-3 | 46 (38) | 28 (42) | 6 (38) | 3 (43) | 9 (28) | |
| ≥4 | 56 (46) | 32 (48) | 7 (44) | 4 (57) | 13 (41) | |
| Treatment received, n (%) | .15 | |||||
| Intensive | 43 (35) | 21 (31) | 8 (50) | 1 (14) | 13 (41) | |
| 3+7 | 20 (16) | 6 (9) | 3 (19) | 1 (14) | 10 (31) | |
| FLAG-IDA | 13 (11) | 9 (13) | 2 (13) | 0 (0) | 2 (6) | |
| NOVE-HIDAC | 10 (8) | 6 (9) | 3 (19) | 0 (0) | 1 (3) | |
| Transplant upfront | 1 (1) | 0 (0) | 0 (0) | 1 (14) | 0 (0) | |
| Nonintensive† | 27 (22) | 13 (19) | 5 (31) | 2 (29) | 7 (22) | |
| BSC | 51 (42) | 33 (49) | 3 (19) | 3 (43) | 12 (38) | |
| Allogeneic transplant, n (%) | .48 | |||||
| Yes | 19 (16) | 9 (14) | 4 (27) | 1 (17) | 5 (16) | |
| No | 103 (84) | 56 (86) | 11 (73) | 5 (83) | 27 (84) |
BCM, below costal margin; BM, bone marrow; ECOG, Eastern Cooperative Oncology Group; ET, essential thrombocythemia; MPAL, mixed-phenotype acute leukemia; LDH, lactate dehydrogenase; MPN-U, MPN unclassifiable; NGS, next-generation sequencing; PET-MF, post–essential thrombocythemia myelofibrosis; PMF, primary myelofibrosis; PPV-MF, post–polycythemia vera myelofibrosis; PV, polycythemia vera.
AML includes 1 patient with myeloid sarcoma.
Nonintensive therapy includes azacitidine (n = 16), clinical trial (n = 10), and low-dose cytarabine (n = 1).