Table 1. Clinical and laboratory features of 722 patients with primary myelofibrosis patients stratified by grades of anemia.
| Variables | All (n=722) | No anemia (n=94) | Mild anemia (n=295) | Moderate anemia (n=98) | Severe anemia (n=235) | P-value univariate | P-value multivariate |
|---|---|---|---|---|---|---|---|
| Age at referral in years median (range) | 64 (22–90) | 60.5 (30–87) | 61 (22–88) | 64.5 (28–89) | 69 (37–90) | <0.0001 | 0.0002 |
| Male (%) | 464 (64%) | 55 (59%) | 192 (65%) | 61 (62%) | 156 (66%) | 0.55 | |
| Leukocytes, × 109/l median (range) | 9 (0.8–236.1) | 13.950 (2.8–4.1) | 9.0 (1.1–176.0) | 6.450 (1.7–236.1) | 7.9 (0.8–218.5) | <0.0001 | 0.061 |
| Platelets, × 109/l median (range) | 212.0 (10.0–466.0) | 309.0 (15.0–288.0) | 243.0 (13.0–255.0) | 202.0 (14.0–282.0) | 140.0 (10.0–2466.0) | <0.0001 | 0.03 |
| Circulating blasts % median (range) | 1 (0–15) | 0 (0–5) | 0 (0–15) | 1 (0–8) | 1 (0–15) | 0.0014 | 0.36 |
| Presence of constitutional symptoms n (%) | 231 (32%) | 20 (21%) | 79 (27%) | 31 (32%) | 101 (43%) | <0.0001 | 0.04 |
| Presence of palpable splenomegaly N evaluable=706 n (%) | 514 (73%) | 63 (68%) | 208 (72%) | 68 (72%) | 175 (75%) | 0.62 | |
| DIPSS-plusa risk N evaluable =703 | <0.0001 | ||||||
| Low | 94 (13%) | 31 (34%) | 61 (21%) | 2 (2%) | 0 (0%) | ||
| Intermediate-1 | 119 (17%) | 27 (29%) | 85 (30%) | 7 (7%) | 0 (0%) | ||
| Intermediate-2 | 259 (37%) | 27 (29%) | 109 (38%) | 62 (64%) | 61 (27%) | ||
| High | 231 (33%) | 7 (8%) | 29 (10%) | 26 (27%) | 169 (73%) | ||
| Driver mutations | <0.0001 | ||||||
| JAK2 mutated n (%) | 476 (66%) | 73 (78%) | 175 (59%) | 66 (67%) | 162 (69%) | 0.03 | |
| CALR type 1/type 1-like n (%) | 115 (16%) | 12 (13%) | 70 (24%) | 14 (14%) | 19 (8%) | 0.01 | |
| CALR type 2/type 2-like n (%) | 24 (3%) | 1 (1%) | 14 (5%) | 5 (5%) | 4(2%) | ||
| MPL mutated n (%) | 38 (5%) | 0 (0%) | 18 (6%) | 6 (6%) | 14 (6%) | ||
| Triple negative n (%) | 69(10%) | 8 (8%) | 18 (6%) | 7 (7%) | 36 (15%) | ||
| Cytogenetic categories N evaluable =703 (97%) | |||||||
| Normal | 426 (61%) | 64 (70%) | 173 (61%) | 56 (58%) | 133 (58%) | ||
| Normal vs abnormal | 0.24 | ||||||
| Favorable | 620 (88%) | 82 (89%) | 255 (90%) | 87 (90%) | 196 (85%) | ||
| Favorable vs unfavorable | 0.40 | ||||||
| ASXL1 N evaluable=480 | 181(38%) | 21 (31%) | 70 (36%) | 28 (42%) | 62 (41%) | 0.50 | |
| SF3B1 N evaluable=415 | 35 (8%) | 4 (7%) | 11 (7%) | 6 (10%) | 14 (10%) | 0.64 | |
| U2AF1 N evaluable=457 | 72 (16%) | 2 (3%) | 15 (8%) | 11 (18%) | 44 (30%) | <0.0001 | <0.0001 |
| SRSF2 N evaluable=474 | 70 (15%) | 9 (13%) | 24 (13%) | 6 (9%) | 31 (20%) | 0.09 | |
| TET2 N evaluable=180 | 32 (18%) | 2 (7%) | 13 (19%) | 7 (30%) | 10 (16%) | 0.21 | |
| EZH2 N evaluable=374 | 16 (4%) | 2 (4%) | 8 (5%) | 2 (4%) | 4 (3%) | 0.85 | |
| ZRSR2 N evaluable=180 | 19 (11%) | 1 (4%) | 11 (16%) | 0 (0%) | 7 (11%) | 0.11 | |
| IDH1 N evaluable=187 | 9 (5%) | 1 (4%) | 3 (4%) | 2 (8%) | 3 (5%) | 0.87 | |
| IDH2 N evaluable=187 | 14 (7%) | 1 (4%) | 5 (7%) | 1 (4%) | 7 (11%) | 0.58 | |
Abbreviation: DIPSS-plus, dynamic international prognostic scoring system-plus.
a
DIPSS-plus:5 DIPSS-plus uses eight independent predictors of inferior survival: age >65 years, hemoglobin <10 g/dl, leukocytes >25 × 109/l, circulating blasts ⩾1%, constitutional symptoms, red cell transfusion dependency, platelet count <100x109/l and unfavorable karyotype (that is, complex karyotype or sole or two abnormalities that include þ8, −7/7q−, i(17q), inv,3 −5/5q−, 12p− or 11q23 rearrangement). The presence of 0, 1, ‘2 or 3' and 4 adverse factors defines low, intermediate-1, intermediate-2 and high-risk disease. Statistically significant P-values are in bold.