Table 1

Hematologic and clinical characteristics of patients stratified according to EZH2 mutational status

PMF
PPPV/PET-MF
P
EZH2
EZH2
Wild-typeMutatedWild-typeMutated
348 22  141  
Median follow-up, mo, (range) (n = 500)* 39.567 (1-340) 28.183 (8-183) .365 29.7 (1-234) 16.8 (1-70) .256 
Median age, y (range) 60.0 (14-90) 66.0 (38-90) .135 62.0 (32-84) 62.0 (52-78) .731 
Male sex, no. (%) (n = 303)* 221 (60.6) 15 (68.2) .481 73 (51.8) 4 (57.1) .781 
Leukocytes, × 106/L, mean ± SD (n = 500)* 12.3 ± 13.3 18.7 ± 11.5 .001 14.3 ± 14.1 13.8 ± 7.0 .360 
Hb, g/L, mean ± SD (n = 497)* 114 ± 27 112 ± 19 .700 114 ± 26 100 ± 18 .153 
Platelets, × 106/L, mean ± SD, (n = 501)* 341.0 ± 345.6 405.0 ± 258.7 .831 404.1 ± 317.6 232.1 ± 198.0 .097 
Peripheral blast cells, %, mean ± SD (n = 329)* 0.7 ± 2.1 1.6 ± 2.1 .002 0.5 ± 2.0 0.4 ± 0.9 .959 
Constitutional symptoms, no. (%) (n = 333)* 67 (28.2) 8 (42.1) .198 34 (47.9) 2 (40.0) .733 
Splenomegaly, no. (%) (n = 494)*   .016   .059 
    0 91 (27.6) 1 (4.5)  17 (12.6) 3 (42.9)  
    1 141 (42.7) 9 (40.9)  58 (43.0) 1 (14.3)  
    2 98 (29.7) 12 (54.5)  60 (44.4) 3 (42.9)  
Abnormal karyotype, no. (%) (n = 195)* 26 (20.2) 4 (36.4) .209 17 (32.7) 2 (66.7) .229 
Unfavorable karyotype, no. (%) (n = 188)* 18 (14.3) 3 (27.3) .252 11 (22.9) 2 (66.7) .092 
JAK2V617F, no. (%) (n = 518)* 204 (58.8) 9 (40.9) .100 105 (74.5) 3 (42.9) .066 
JAK2V617F allele burden, %, mean ± SD 45.7 ± 22.9 42.2 ± 22.1 .603 64.1 ± 23.4 61.1 ± 52.6 .747 
MPL W515L/K, no. (%) (n = 466)* 13 (3.9) 0 (0.0) .344 5 (4.7) 0 (0.0) .586 
IWG-MRT score, no. (%) (n = 192)*   .002   
    Low 55 (31.8) 4 (21.1)     
    Int-1 44 (25.4) 5 (26.3)     
    Int-2 43 (24.9) 0 (0.0)     
    High 31 (17.9) 10 (52.6)     
Progression to acute leukemia, no. (%) (n = 443)* 57 (17.6) 7 (31.8) .098 16 (17.2) 1 (20.0) .872 
Death, no. (%) (n = 494)* 86 (26.0) 13 (61.9) < .001 28 (20.6) 1 (16.7) .816 
PMF
PPPV/PET-MF
P
EZH2
EZH2
Wild-typeMutatedWild-typeMutated
348 22  141  
Median follow-up, mo, (range) (n = 500)* 39.567 (1-340) 28.183 (8-183) .365 29.7 (1-234) 16.8 (1-70) .256 
Median age, y (range) 60.0 (14-90) 66.0 (38-90) .135 62.0 (32-84) 62.0 (52-78) .731 
Male sex, no. (%) (n = 303)* 221 (60.6) 15 (68.2) .481 73 (51.8) 4 (57.1) .781 
Leukocytes, × 106/L, mean ± SD (n = 500)* 12.3 ± 13.3 18.7 ± 11.5 .001 14.3 ± 14.1 13.8 ± 7.0 .360 
Hb, g/L, mean ± SD (n = 497)* 114 ± 27 112 ± 19 .700 114 ± 26 100 ± 18 .153 
Platelets, × 106/L, mean ± SD, (n = 501)* 341.0 ± 345.6 405.0 ± 258.7 .831 404.1 ± 317.6 232.1 ± 198.0 .097 
Peripheral blast cells, %, mean ± SD (n = 329)* 0.7 ± 2.1 1.6 ± 2.1 .002 0.5 ± 2.0 0.4 ± 0.9 .959 
Constitutional symptoms, no. (%) (n = 333)* 67 (28.2) 8 (42.1) .198 34 (47.9) 2 (40.0) .733 
Splenomegaly, no. (%) (n = 494)*   .016   .059 
    0 91 (27.6) 1 (4.5)  17 (12.6) 3 (42.9)  
    1 141 (42.7) 9 (40.9)  58 (43.0) 1 (14.3)  
    2 98 (29.7) 12 (54.5)  60 (44.4) 3 (42.9)  
Abnormal karyotype, no. (%) (n = 195)* 26 (20.2) 4 (36.4) .209 17 (32.7) 2 (66.7) .229 
Unfavorable karyotype, no. (%) (n = 188)* 18 (14.3) 3 (27.3) .252 11 (22.9) 2 (66.7) .092 
JAK2V617F, no. (%) (n = 518)* 204 (58.8) 9 (40.9) .100 105 (74.5) 3 (42.9) .066 
JAK2V617F allele burden, %, mean ± SD 45.7 ± 22.9 42.2 ± 22.1 .603 64.1 ± 23.4 61.1 ± 52.6 .747 
MPL W515L/K, no. (%) (n = 466)* 13 (3.9) 0 (0.0) .344 5 (4.7) 0 (0.0) .586 
IWG-MRT score, no. (%) (n = 192)*   .002   
    Low 55 (31.8) 4 (21.1)     
    Int-1 44 (25.4) 5 (26.3)     
    Int-2 43 (24.9) 0 (0.0)     
    High 31 (17.9) 10 (52.6)     
Progression to acute leukemia, no. (%) (n = 443)* 57 (17.6) 7 (31.8) .098 16 (17.2) 1 (20.0) .872 
Death, no. (%) (n = 494)* 86 (26.0) 13 (61.9) < .001 28 (20.6) 1 (16.7) .816 

P values in bold indicate statistical significance.

*

Number of patients for whom information was available.

Splenomegaly: 0 = not palpable; 1 = palpable at < 10 cm from left costal margin; and 2 = palpable at > 10 cm from left costal margin.

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