Table 1 Clinical and laboratory features of 1002 patients with primary myelofibrosis, stratified by normal vs abnormal karyotype and the most frequent sole abnormalities

From: Revised cytogenetic risk stratification in primary myelofibrosis: analysis based on 1002 informative patients

Variables

All patients (n = 1002)

Normal karyotype (n = 553; 55%)

Abnormal karyotype (n = 449; 45%)

P valueb

Sole 20q− (n = 74; 7.4%)

Sole 13q− (n = 56; 5.6%)

Sole +8 (n = 26; 2.6%)

Sole +9 (n = 14; 1.4%)

Pvaluec

Age in years; median (range)

65 (19–92)

65 (19–89)

65 (30–92)

0.02

69 (30–83)

63 (37–87)

68 (30–85)

69 (46–80)

0.03

Age >65 years; n (%)

523 (52)

277 (50)

246 (55)

0.13

46 (65)

24 (43)

16 (62)

10 (71)

0.03

Males; n (%)

625 (62)

337 (61)

288 (64)

0.3

50 (70)

33 (59)

14 (54)

9 (64)

0.5

Hemoglobin, g/dl; median (range)

10 (5–16.7)

10.3 (5–16.1)

10 (5.2–16.7)

0.001

9.9 (6.7–15)

11 (6.6–14.9)

10 (6.2–13)

11.2 (7.8–14)

0.1

Hemoglobin <10 g/dl; n (%)

514 (51)

260 (47)

254 (57)

0.003

45 (64)

23 (41)

16 (62)

6 (43)

0.04

Transfusion -requiring; n (%)

367 (37)

186 (34)

181(40)

0.03

45 (64)

40 (71)

14 (54)

9 (64)

0.6

Leukocytes, x 109/l; median (range)

9 (1–236.1)

9.9 (1–236.1)

8 (1–218.5)

0.007

6.1 (1–71.5)

10 (2.2–176)

7 (1.3–142)

10.9 (2.6–40)

0.001

Leukocytes >25 × 109/l; n (%)

162 (16)

89 (16)

73 (16)

0.9

6 (8)

12 (21)

3 (12)

2 (14)

0.3

Platelets, x 109/l; median (range)

204.5 (6–2466)

245 (8–2466)

153 (6–2282)

<0.001

159 (12–1921)

246 (14–1043)

158 (17–684)

172 (38–769)

<0.001

Platelets <100 × 109/l; n (%)

259 (26)

108 (20)

151 (34)

<0.001

23 (32)

8 (14)

9 (35)

4 (29)

0.03

Circulating blast %; median (range)

1 (0–18)

1 (0–15)

1 (0–18)

0.001

0 (0–6)

1 (0–13)

1 (0–18)

0 (0–4)

0.2

Circulating blasts ≥1%; n (%)

538 (54)

279 (50)

259 (58)

0.02

29 (41)

32 (57)

14 (54)

6 (43)

0.4

Constitutional symptoms; n (%)

333 (33)

177 (32)

156 (35)

0.4

23 (32)

13 (23)

7 (27)

7 (50)

0.4

DIPSSa risk Distribution

   

0.002

    

0.2

High; n (%)

112 (11)

54 (10)

58 (13)

8 (11)

3 (5)

2 (8)

0 (0)

Intermediate-2; n (%)

431 (43)

217 (39)

214 (48)

38 (54)

24 (43)

13 (50)

8 (57)

Intermediate-1; n (%)

334 (33)

99 (36)

135 (30)

17 (24)

20 (36)

11 (42)

5 (36)

Low; n (%)

125 (13)

83 (15)

42 (9)

8 (11)

9 (16)

0 (0)

1 (7)

Driver mutational status ‘N’evaluable = 637

   

0.6

    

0.1

JAK2; n (%)

419 (66)

235 (64)

184 (68)

34 (81)

16 (50)

7 (78)

11 (100)

CALR type 1/like; n (%)

100 (15)

58 (16)

42 (16)

2 (5)

10 (31)

0 (0)

0 (0)

CALR type 2/like; n (%)

23 (4)

16 (4)

7 (3)

1 (2)

1 (3)

0 (0)

0 (0)

MPL; n (%)

33 (5)

21 (6)

12 (4)

2 (5)

3 (10)

0 (0)

0 (0)

Triple-negative; n (%)

62 (10)

39 (10)

23 (9)

3 (7)

2 (6)

2 (22)

0 (0)

ASXL1-mutated; n (%) ‘N’ evaluable = 436

165 (38)

108 (43)

57 (31)

0.01

5 (20)

3 (12)

1 (14)

2 (25)

0.004

SRSF2-mutated; n (%) ‘N’ evaluable = 423

61 (14)

36 (15)

25 (14)

0.8

7 (29)

1 (4)

1 (13)

1 (13)

0.2

  1. a DIPSS, Dynamic International Prognostic Scoring System-plus uses five independent predictors of inferior survival: age >65 years, hemoglobin <10 g/dL, leukocytes >25 × 109/L, circulating blasts ≥1% and constitutional symptoms
  2. b P value for comparison of normal vs abnormal karyotype
  3. c P value for comparison of five groups: normal karyotype vs sole abnormalities of 20q−, 13q−, +8 and +9
  4. Bold indicates significant differences
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