Table 1 Clinical and laboratory features of 277 patients with primary myelofibrosis (Mayo cohort), stratified by the presence or absence of CALR and ASXL1 mutations

From: CALR and ASXL1 mutations-based molecular prognostication in primary myelofibrosis: an international study of 570 patients

Variables All patients (n=277) CALR−ASXL1+ (n=62; 22%) CALR− ASXL1− (n=146; 53%) CALR+ ASXL1+ (n=23; 8%) CALR+ ASXL1− (n=46; 17%) P-value
Age in years, median (range) 64 (32–87) 68 (39–81) 65 (35–87) 57 (42–70) 56 (32–82) <0.0001
Age >65 years, n (%) 119 (43%) 40 (64.5%) 65 (44.5%) 4 (17.4%) 10 (21.7%) <0.0001
Males (%) 177 (64.6%) 44 (71%) 89 (61%) 19 (82.6%) 25 (54.3%) 0.06
Hemoglobin, g/dl; median (range) 10.4 (5.8–16.1) 10.0 (6.6–16.1) 10.3 (5.8–16.0) 10.6 (6.5–12.9) 11.3 (8.1–14.3) 0.01
Leukocytes, × 109/l; median (range) 9.0 (1.0–218) 11.9 (1.9–146) 9.4 (1.0–218) 8.0 (1.8–26.5) 7.7 (3.5–44.0) 0.03
Platelets, × 109/l; median (range) 240 (11–2466) 211 (11–1288) 208 (12–2466) 275 (76–563) 415 (57–1493) <0.0001
Circulating blast %; median (range) 1 (0–15) 1 (0–11) 0 (0–14) 1 (0–15) 1 (0–12) 0.03
DIPSS-plus a risk group
 Low 34 (12.2%) 4 (6.5%) 14 (9.6%) 2 (8.7%) 14 (30.4%) <0.0001
 Intermediate-1 103 (37.2%) 22 (35.5%) 59 (40.4%) 10 (43.5%) 12 (26.1%)  
 Intermediate-2 54 (19.5%) 4 (6.5%) 30 (20.5%) 5 (21.7%) 15 (32.6%)  
 High 86 (31%) 32 (51.5%) 43 (29.5%) 6 (26.1%) 5 (10.9%)  
Constitutional symptoms, n (%) 94 (33.9%) 33 (53.2%) 44 (30.1%) 10 (43.5%) 7 (15.2%) 0.0002
Circulating blasts 1%, n (%) 128 (46.2%) 26 (41.9%) 77 (52%) 4 (17.4%) 22 (47.8%) 0.02
Hemoglobin <10 g/dl, n (%) 131 (47.3%) 35 (56.4%) 76 (52%) 10 (43.5%) 10 (21.7%) 0.001
Transfusion requiring, n (%) 88 (31.8%) 26 (41.9%) 53 (36.3%) 5 (21.7%) 4 (8.7%) 0.0008
Leukocytes >25 × 109/l, n (%) 43 (15.5%) 18 (29.0%) 19 (13%) 2 (8.7%) 4 (8.7%) 0.008
Leukocytes >10 × 109/l, n (%) 119 (43%) 34 (54.8%) 66 (45.2%) 8 (34.8%) 11 (23.9%) 0.01
Platelets <100 × 109/l, n (%) 57 (20.6%) 19 (30.6%) 32 (21.9%) 1 (4.3%) 5 (10.9%) 0.02
Platelets >450 × 109/l, n (%) 51 (18.4%) 10 (16.4%) 19 (13%) 3 (13%) 19 (41.3%) 0.0002
IDH1/2-mutated, n (%) ‘N’ evaluable=266 13 (4.9%) 4 (6.7%) 4 (2.9%) 1 (4.5%) 4 (8.7%) 0.39
SF3B1-mutated, n (%) ‘N’ evaluable=254 18 (7.1%) 2 (3.3%) 15 (11.5%) 0 (0%) 1 (2.4%) 0.04
U2AF1-mutated; n (%) ‘N’ evaluable= 245 39 (15.9%) 18 (30.5%) 20 (15.5%) 0 (0%) 1 (2.6%) 0.0004
SRSF2-mutated, n (%) ‘N’ evaluable=266 30 (11.3%) 11 (18.0%) 17 (12.4%) 0 (0%) 2 (4.3%) 0.05
U2AF1/SRSF2/SF3B1 mutated ‘N’ evaluable=240; n(%) 81 (33.7%) 29 (49.1%) 48 (38.1%) 0 (0%) 4 (10.2%) <0.0001
EZH2-mutated, n (%) ‘N’ evaluable=251 14 (5.6%) 4 (6.7%) 6 (4.7%) 1 (5%) 3 (7%) 0.9
Cytogenetic categories, n (%) ‘N’ evaluable=274
 Normal 177 (64.6%) 49 (80.3%) 82 (56.6%) 16 (69.6%) 30 (66.7%) 0.01
 Abnormal 97 (35/4%) 12 (19.7%) 63 (43.4%) 7 (30.4%) 15 (33.3%)  
Cytogenetic categories, n (%) ‘N’ evaluable=274       0.62
 Favorable 248 (90.5%) 57 (93.4%) 129 (89%) 20 (87%) 42 (93.3%)  
 Unfavorable 26 (9.5%) 4 (6.6%) 16 (11%) 3 (13%) 3(6.7%)  
Deaths, n (%) 189 (68%) 57 (92%) 101 (69%) 12 (52%) 19 (41%) <0.0001
Documented leukemic transformations, n (%) 31 (11%) 9 (15%) 16 (11%) 1 (4%) 5 (11%) 0.7
  1. Abbreviation: DIPSS, Dynamic International Prognostic Scoring System.
  2. The presence of 0, 1, ‘2 or 3’ and 4 adverse factors defines low, intermediate-1, intermediate-2 and high-risk disease.
  3. aDIPSS-plus, Dynamic International Prognostic Scoring System-plus (reference in text): 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 <100 × 109/l and unfavorable karyotype (that is, complex karyotype or sole or two abnormalities that include +8, −7/7q-, i(17q), inv(3), -5/5q-, 12p- or 11q23 rearrangement).