Table 1 Clinicopathologic features of the study group.
Primary myelofibrosis (N = 14) | Polycythemia vera (N = 2) | Post-ET/PV myelofibrosis (N = 2) | Chronic myelomonocytic leukemia (N = 3) | Acute myeloid leukemia (N = 4) | MPN-U (N = 1) | MDS/MPN-U (N = 1) | |
|---|---|---|---|---|---|---|---|
Male: female | 12:2 | 2:0 | 2:0 | 2:1 | 3:1 | 0:1 | 1:0 |
Median age (range) | 73.8 (56.3–85.2) | 72.65 (71 and 74.4) | 76.34 (74.2 and 78.5) | 76.36 (66.8–79.4) | 70.87 (64.6–77) | 72.8 | 79.7 |
Splenomegaly n (%) | 8/14 (57%) | 0/2 (0%) | 1/2 (50%) | 2/3 (66.6%) | 3/4 (75%) | 1/1 (100%) | 0/1 (0%) |
Hb (g/dL), median (range) | 9.4 (5.5–14.6) | 11.7 (10 and 13.4) | 8.85 (8.4 and 9.3) | 9.1 (8.7–10.5) | 10.55 (9.5–12.1) | 13.2 | 7.4 |
Transfusion dependence n (%) | 4/14 (28.6%) | 0/2 (0%) | 1/2 (50%) | 0/3 (0%) | 0/4 (0%) | 0/1 (0%) | 1/1 (100%) |
ANC (×109/L), median (range) | 9.71 (3–22.4) | 11.88 (4.8 and 19) | 1.7 (0.3 and 3.1) | 17.56 (10.1–20.1) | 0.65 (0.1–11.3) | 29.7 | 0.88 |
AMC (×109/L), median (range) | 0.945 (0–7.6) | 0.90 (0.3 and 1.5) | 0.06 (0 and 0.1) | 1.58 (1–17.6) | 0.26 (0.02–7.4) | 6.5 | 0.2 |
Platelets (×109/L), median (range) | 107.5 (3–448) | 903 (676 and 1,130) | 500 (279 and 721) | 127 (82–256) | 46.50 (15–241) | 124 | 42 |
Ring sideroblasts n (RS %) | 1/14 (35%) | 0/2 (0%) | 0/2 (0%) | 0/3 (0%) | 0/4 (0%) | 0/1 (0%) | 1/1 (48%) |
Erythroid dysplasia n (%) | 3/14 (21.4%) | 0/2 (0%) | 0/2 (0%) | 1/3 (33.3%) | 1/4 (25%) | 0/1 (0%) | 1/1 (100%) |
Granulocytic dysplasia n (%) | 5/14 (35.7%) | 0/2 (0%) | 0/2 (0%) | 2/3 (66.6%) | 0/4 (0%) | 0/1 (0%) | 1/1 (100%) |
Megakaryocytes n (%) | |||||||
MDS-like | 1/14 (7.1%) | 0 | 0 | 1/3 (33.3%) | 1/4 (25%) | 0 | 1/1 (100%) |
MPN-like | 2/14 (14.3%) | 1/2 (50%) | 0 | 0 | 0/4 (0%) | 0 | 0 |
Hybrid MDS/MPN-like | 9/14 (64.3%) | 1/2 (50%) | 1/2 (50%) | 0 | 1/4 (25%) | 1/1 (100%) | 0 |
Unremarkable | 0 | 0 | 0 | 1/3 (33.3%) | 0 | 0 | 0 |
Too few to assess | 2/14 (14.3%) | 0 | 1/2 (50%) | 1/3 (33.3%) | 2/4 (50%) | 0 | 0 |
BM fibrosis MF2–3 n (%) | 12/14 (85.7%) | 1/2 (50%) | 2/2 (100%) | 2/3 (66.6%) | not available | 0/1 (0%) | not available |
Abnormal karyotype n (%) | 6/14 (42.85%) | 0/2 (0%) | 0/2 (0%) | 1/3 (33.3%) | 3/4 (75%) | 0/1 (0%) | 1/1 (100%) |
Complex karyotype/−5 n (%) | 4/14 (28.6%) | 0/2 (0%) | 0/2 (0%) | 0/3 (0%) | 1/4 (25%) | 0/1 (0%) | 0/1 (0%) |
MPN-driver mutation, Median VAF (range) | JAK2 10/14 38% (6–63) MPL 4/14 45% (28–88) | JAK2 2/2 31% (20 and 43) | JAK2 1/2 23% CALR 1/2 58% | JAK2 2/3 36% (11 and 60) MPL 1/3 32% | JAK2 2/4 25 (15 and 35) MPL 3/4 11 (6–20) | JAK2 1/1 73% | JAK2 1/1 2% |
SRSF2 mutation, median VAF (range) | 44% (2–65) | 26% (24 and 47) | 27% (5 and 49) | 44% (36–48%) | 47% (32–52) | 51% | 42% |
AML progression n (progression time from initial diagnosis, months) | 1/14 (34.67) | 1/2 (20.53) | 0/2 (0%) | 0/3 (0%) | 2/4 (36.3 and 296.7) | 0/1 (0%) | 0/1 (0%) |
Median OS months (range) | 41.81 (9.9–82.3) | 24.27 (23.5 and 25.03) | 51.98 (45.33 and 58.63) | 47.43 (35.82–47.55) | 35.2 (9.73–313.47) | 20.4 | 19.73 |
NGS performed at the time of initial diagnosis n (%) | 0/14 (0%) | 2/2 (100%) | 0/2 (0%) | 0/3 (0%) | 0/4 (0%) | 0/1 (0%) | 0/1 (0%) |
Months between initial diagnosis and NGS, median (range) | 14/14 (100%) 18.69 (0.62–80.61) | N/A | 2/2 (100%) 37.17 (18.81 and 55.53) | 3/3 (100%) 36.2 (12.62–66.44) | 4/4 (100%) 34.88 (7.13–292.6) | 1/1(100%) 1.38 | 1/1 (100%) 14.7 |
Previous treatment (other than cytoreduction) received prior to NGS | 8/14 (57%) | 0/2 | 2/2 (100%) | 3/3 (100%) | 2/4 (50%) | 1/1 (100%) | 1/1 (100%) |
