Table 2. Comparison of clinical characteristics of patients with primary myelofibrosis, who were evaluated within 1 year of diagnosis, stratified by the presence or absence of IDH, MPL and JAK2 mutations.
Variables | All patients (n=178) | IDH mutated (n=10) | MPL mutated (n=9) | JAK2 mutated (n=96) | IDH/MPL/JAK2 un-mutated (n=63) | P-value |
---|---|---|---|---|---|---|
Age (years); median (range) | 63 (14–81) | 66 (50–74) | 60 (35–66) | 65 (28–81) | 58 (14–79) | 0.005 |
Age >65 years; n (%) | 70 (39%) | 5 (50%) | 1 (11%) | 48 (50%) | 16 (25%) | 0.004 |
Males (%) | 116 (65%) | 7 (70%) | 7 (80%) | 63 (66%) | 39 (62%) | 0.79 |
Hemoglobin, g/dl; median (range) | 10 (6–15) | 11 (7–15) | 10 (6–14) | 10 (7–15) | 11 (6–14) | 0.50 |
Leukocyte count, × 109/l; median (range) | 8 (1–147) | 10 (4–48) | 11 (4–50) | 9 (1–99) | 7 (2–147) | 0.15 |
Platelet count, × 109/l; median (range) | 253 (12–1493) | 159 (79–410) | 146 (31–662) | 245 (12–984) | 316 (14–1493) | 0.13 |
DIPSS-plus risk group (%) | ||||||
Low | 25 (14%) | 0 | 2 (22%) | 13 (14%) | 10 (16%) | 0.60 |
Intermediate-1 | 36 (20%) | 2 (20%) | 1 (11%) | 15 (16%) | 18 (29%) | |
Intermediate-2 | 62 (35%) | 4 (40%) | 3 (33%) | 35 (36%) | 20 (32%) | |
High | 55(31%) | 4 (40%) | 3 (33%) | 33 (34%) | 15 (24%) | |
Constitutional symptoms; n (%) | 65 (37%) | 4 (40%) | 2 (22%) | 38 (40%) | 21 (33%) | 0.68 |
Circulating blasts ⩾1% n (%) | 101 (57%) | 8 (80%) | 5 (56%) | 54 (56%) | 34(53%) | 0.49 |
Hemoglobin <10 g/dl; n (%) | 77 (43%) | 4 (40%) | 6 (67%) | 47 (49%) | 20 (32%) | 0.08 |
Leukocytes >25 × 109/l; n (%) | 26 (15%) | 2 (20%) | 2 (22%) | 14 (15%) | 8 (13%) | 0.84 |
Platelets <100 × 109/l; n (%) | 36 (20%) | 1 (10%) | 4 (44%) | 17 (18%) | 14 (22%) | 0.22 |
Leukocytes <4 × 109/l; n (%) | 27 (15%) | 1 (10%) | 1 (11%) | 12 (13%) | 13 (21%) | 0.51 |
Palpable spleen >10 cm; n (%) | 44 (25%) | 2 (20%) | 2 (22%) | 28 (29%) | 12 (19%) | 0.45 |
Splenectomy; n (%) | 24 (13%) | 1 (10%) | 3 (33%) | 12 (13%) | 8 (13%) | 0.35 |
Cytogenetic categories | ||||||
Normal | 116 (65%) | 7 (70%) | 6 (67%) | 58 (60%) | 45 (71%) | 0.75 |
Favorable | 48 (27%) | 2 (20%) | 3 (33%) | 30 (31%) | 13 (21%) | |
Unfavorable | 14 (8%) | 1 (10%) | 0 (0%) | 8 (8%) | 5 (8%) | |
Transplanted; n (%) | 15 (8%) | 0 (0%) | 2 (22%) | 5 (5%) | 8 (13%) | 0.12 |
Deaths; n (%) | 107 (60%) | 9 (90%) | 6 (67%) | 59 (61%) | 32 (51%) | 0.09 |
Leukemic transformations; n (%) | 22 (12%) | 4 (40%) | 1 (11%) | 13 (14%) | 4 (6%) | 0.03 |
Abbreviation: DIPSS-plus, Dynamic International Prognostic Scoring System-Plus.
Bold values indicate significant differences.