Table 2 Clinical and laboratory characteristics of 641 patients with primary myelofibrosis stratified by the revised cytogenetic risk modela

From: GIPSS: genetically inspired prognostic scoring system for primary myelofibrosis

Variables

All patients (n = 641)b

Very high risk karyotype (n = 43)

Unfavorable karyotype (n = 94)

Favorable karyotype (n = 504)

P-value

Age in years; median (range)

63 (19–89)

65 (46–87)

64 (38–81)

62 (19–89)

0.02

Age >65 years; n (%)

263 (41)

22 (51)

38 (40)

203 (40)

0.4

Males (%)

411 (64)

26 (60)

67 (71)

318 (63)

0.3

Hemoglobin <10 g/dl; n (%)

260 (41)

28 (65)

42 (45)

190 (38)

0.001

Transfusion requiring; n (%)

191 (30)

25 (58)

30 (32)

136 (27)

<0.001

Leukocytes, x109/l; median (range)

9 (1–219)

10 (2–75)

8 (1.4–219)

9.2 (1–176)

0.5

Leukocytes >25 × 109/l; n (%)

89 (14)

9 (23)

14 (15)

66 (13)

0.2

Platelets, x109/l; median (range)

237 (10–2466)

123.5 (11–1000)

154 (10–2282)

261 (12–2466)

<0.001

Platelets <100 × 109/l; n (%)

122 (19)

18 (45)

24 (26)

80 (16)

<0.001

Circulating blasts ≥1%; n (%)

297 (47)

29 (71)

49 (54)

219 (44)

0.001

Circulating blasts ≥2%; n (%)

173 (27)

23 (53)

29 (31)

121 (24)

<0.001

Constitutional symptoms; n (%)

208 (32)

19 (44)

36 (38)

153 (30)

0.07

DIPSS c risk distribution

    

<0.001

 High; n (%)

83 (13)

14 (33)

14 (15)

55 (11)

 

 Intermediate-2; n (%)

242 (38)

24 (56)

36 (38)

182 (36)

 

 Intermediate-1 n (%)

214 (33)

3 (7)

35 (37)

176 (35)

 

 Low; n (%)

102 (16)

2 (4)

9 (10)

91 (18)

 

Driver mutations

    

0.14

 JAK2; n (%)

368 (57)

21 (49)

56 (60)

291 (57)

 

 CALR type 1/like; n (%)

123 (19)

6 (14)

23 (24)

94 (19)

 

 CALR type 2/like; n (%)

32 (5)

3 (7)

2 (2)

27 (5)

 

 MPL; n (%)

46 (7)

3 (7)

4 (4)

39 (8)

 

 Triple negative; n (%)

72 (12)

10 (23)

9 (10)

53 (11)

 

ASXL1-mutated; n (%)

242 (38)

24 (56)

35 (37)

183 (36)

0.04

SRSF2-mutated; n (%)

89 (14)

12 (28)

9 (10)

68 (13)

0.01

U2AF1Q157-mutated; n (%)

50 (8)

2 (5)

7 (7)

41 (8)

0.7

EZH2-mutated; n (%)

37 (7)

3 (8)

4 (5)

30 (7)

0.7

IDH1/2-mutated; n (%)

23 (4)

2 (5)

3 (4)

18 (4)

0.9

MIPSS70-plus d risk distribution

    

<0.001

 Very high; n (%)

76 (12)

33 (77)

37 (39)

6 (1)

 

 High; n (%)

263 (41)

10 (23)

53 (57)

200 (40)

 

 Intermediate; n (%)

125 (20)

0 (0)

4 (4)

121 (24)

 

 Low; n (%)

177 (27)

0 (0)

0 (0)

177 (35)

 
  1. The values in bold indicate a significant P-value (<0.05)
  2. ASXL1 additional sex combs like 1, SRSF2 serine/arginine-rich splicing factor 2, U2AF1 U2small nuclear RNA auxiliary factor 1, EZH2 enhancer of zeste homolog 2, IDH1/2 isocitrate dehydrogenase 1/2, JAK2 Janus kinase 2, CALR calreticulin, MPL myeloproliferative leukemia virus oncogene
  3. a Revised cytogenetic risk stratification: “very high risk (VHR)”—single/multiple abnormalities of −7, i(17q), inv(3)/3q21, 12p−/12p11.2, 11q−/11q23, +21, or other autosomal trisomies, not including +8/+9; “favorable”—normal karyotype or sole abnormalities of 13q−, +9, 20q−, chromosome 1 translocation/duplication or sex chromosome abnormality including—Y; “unfavorable”—all other abnormalities (reference in the text)
  4. b In most instances, including all GIPSS-relevant variables, information was available in all 641 patients. In all instances of genetic risk factor analysis, a minimum of 500 informative cases was required and missing information did not exceed 10%
  5. c DIPSS, Dynamic International Prognostic Scoring System uses five independent predictors of inferior survival: age >65 years, hemoglobin <10 g/dl, leukocytes >25 × 109/L, circulating blasts ≥1% and constitutional symptoms (reference in the text)
  6. d MIPSS70-plus, Mutation-Enhanced International Prognostic Score System for transplant-age patients uses: hemoglobin <10 g/dl, leukocytes >25 × 109/L, platelets <100 × 109/L, circulating blasts ≥2%, constitutional symptoms, absence of CALR type 1 mutation, presence of high-molecular risk mutation (e.g., ASXL1, EZH2, SRSF2, IDH1/2), presence of two or more high-molecular risk mutations and a two-tiered revised cytogenetic risk stratification where very high risk and unfavorable karyotype are grouped together as “unfavorable” (reference in the text)
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