Table 1.
Baseline characteristics
Variables | Median [range] or n (%) | ||
---|---|---|---|
MPN-AML (n=21) | MPN-AP (n= 13) | High risk DIPSS plus PMF (n= 11) | |
Age in years | 64 [45–82] | 63 [50–81] | 67 [55–77] |
Gender | |||
Male | 16 (76) | 8 (62) | 8 (73) |
Female | 5 (24) | 5 (38) | 3 (27) |
Initial MPN | |||
ET | 4 (19) | 2 (15) | 0 (0) |
PV | 5 (24) | 5 (39) | 0 (0) |
PMF | 10 (48) | 6 (46) | 11 (100) |
MPNu | 2 (10) | 0 (0) | 0 (0) |
WBC (109/L) | 8 [1–55] | 11.6 [1–50] | 41.5 [2–140] |
Hb (g/dl) | 9 [8.3–11.5] | 9.5 [6–11.7] | 9.2 [7.7–11.7] |
Plt (109/L) | 39 [3–1912] | 73 [14–267] | 69 [9–860] |
Bone marrow blasts (%) | 31 [21–81] | 14 [10–17] | 2 [0–9] |
JAK2V617F mutation | 11 (52) | 8 (62) | 7 (64) |
FLT3 ITDa | 1 (5) | 1 (8) | 0 |
RASb | 0 | 0 | 2 (25) |
IDH1/2c | 0 | 2 (28) | 0 |
DNMT3Ad | 0 | 0 | 0 |
ASXL1e | 0 | 0 | 0 |
NPM-1f | 0 | 1 (14) | 0 |
EZH2g | 0 | 0 | 0 |
TET2h | 0 | 0 | 0 |
SRSF2i | 0 | 0 | 0 |
Cytogenetics[1, 3] | |||
Unfavorable* | 12 (57) | 6 (46) | 2 (18) |
No. of prior therapies for MPN | 1 [0–4] | 2 [1–5] | 1 [0–4] |
Time to Decitabine (months) | 8 [0.5–241] | 65 [0–389] | 19 [3–195] |
Decitabine as a: | |||
1st line of therapy | 12 (57) | 1 (8) | 2 (18) |
2nd line of therapy | 8 (38) | 2 (15) | 4 (36) |
3rd or greater line of therapy | 1 (5) | 10 (77) | 5 (45) |
Decitabine alone | 12 (57) | 12 (92) | 5 (45) |
Decitabine + gemtuzumab ozogamicin | 7 (33) | 0 (0) | 5 (45) |
Decitabine + ruxolitinib | 2 (10) | 1 (8) | 1 (10) |
No. decitabine cycles | 2 [1–15] | 2 [1–37] | 3 [1–8] |
Mortality after ≤ 4 weeks of therapy | 0 (0) | 0 (0) | 0 (0) |
DIPSS; dynamic international prognostic scoring system, MPN; myeloproliferative neoplasm, ET; essential thrombocytosis, PV; polycythemia vera, PMF; primary myelofibrosis, AML; acute myeloid leukemia, AP; accelerated phase, WBC; white blood cell, Hb; hemoglobin, Plt; platelet.
Unfavorable: (complex, +8, -7/7q-, i (17q), -5/5q-, 12p-, inv (3), or 11q23).
18 MPN-AML, 12 MPN-AP and 8 PMF patients were evaluated for FLT3 ITD;
12 MPN-AML, 8 MPN-AP and 8 PMF were evaluated for RAS;
12 MPN-AML, 7 MPN-AP and 6 PMF evaluated for IDH1/2;
5 MPN-AML, 5 MPN-AP and 6 PMF were evaluated for ASXL1, DNMT3A, EZH2, TET2 and SRSF2;
10 MPN-AML, 7 MPN-AP and 7 PMF patients were evaluated for NPM-1 mutation.