Table 2

Hematologic and clinical characteristics of EZH2-mutated subjects with PMF stratified according to JAK2V617F mutational status

EZH2 wild-type
PEZH2 mutated
P
JAK2
JAK2
Wild-typeV617FWild-typeV617F
143 204  13  
Follow-up, mo (range) n = 354)* 49.4 (1-340) 37.9 (1-282) .020 25.7 (8-182) 30.7 (11-84) .616 
Median age, y, (range) 55.0 (14-88) 63.0 (18-90) .001 57.0 (41-81) 70 (38-90) .324 
Male sex, no. (%) (n = 369)* 90 (62.9) 120 (58.8) .440 8 (61.5) 7 (77.8) .421 
Leukocytes, × 106/L, mean ± SD (n = 354)* 11.3 ± 13.0 13.1 ± 13.5 .005 16.2 ± 11.5 22.4 ± 11.1 .117 
Hemoglobin, g/L, mean ± SD (n = 352)* 109 ± 24 118 ± 28 .002 107 ± 22 118 ± 14 .171 
Platelet, × 106/L, mean ± SD (n = 355)* 447.0 ± 403.2 408.7 ± 308.0 .840 392.8 ± 255.0 422.5 ± 278.6 .764 
Peripheral blast cells, %, mean ± SD (n = 240)* 1.0 ± 2.6 0.5 ± 1.7 .090 1.4 ± 2.4 1.8 ± 1.8 .299 
Constitutional symptoms, no. (%) (n = 257)* 26 (28.3) 41 (28.1) .976 3 (27.3) 5 (62.5) .125 
Splenomegaly, no. (%) (n = 352)*   .747   .518 
    0 41 (29.7) 50 (26.0)  1 (7.7) 0 (0.0)  
    1 58 (42.0) 83 (43.2)  6 (46.2) 3 (33.3)  
    2 39 (28.3) 59 (30.7)  6 (46.2) 6 (66.7)  
Unfavorable karyotype, no. (%) (n = 137) 8 (12.1) 10 (16.7) .466 2 (28.6) 1 (25.0) .898 
JAK2V617F allele burden, %, mean ± SD  45.9 ± 23.0   42.2 ± 22.1  
MPL W515L/K, no. (%) (n = 354)* 12 (8.8) 1 (0.5) < .0001 0 (0.0) 0 (0.0)  
IWG-MRT score, no. (%) (n = 192)*   .484   .247 
    Low 26 (36.6) 29 (28.4) 3 (27.3) 1 (12.5) 
    Int-1 14 (19.7) 30 (29.4) 4 (36.4) 1 (12.5) 
    Int-2 18 (25.4) 25 (24.5) 0 (0.0) 0 (0.0) 
    High 13 (18.3) 18 (17.6) 4 (36.4) 6 (75.0) 
Progression to acute leukemia, no. (%) (n = 345)* 26 (19.3) 31 (16.5) .520 6 (46.2) 1 (11.1) .083 
Death, no. (%) (n = 352)* 38 (27.7) 48 (24.7) .541 7 (53.8) 6 (75.0) .332 
EZH2 wild-type
PEZH2 mutated
P
JAK2
JAK2
Wild-typeV617FWild-typeV617F
143 204  13  
Follow-up, mo (range) n = 354)* 49.4 (1-340) 37.9 (1-282) .020 25.7 (8-182) 30.7 (11-84) .616 
Median age, y, (range) 55.0 (14-88) 63.0 (18-90) .001 57.0 (41-81) 70 (38-90) .324 
Male sex, no. (%) (n = 369)* 90 (62.9) 120 (58.8) .440 8 (61.5) 7 (77.8) .421 
Leukocytes, × 106/L, mean ± SD (n = 354)* 11.3 ± 13.0 13.1 ± 13.5 .005 16.2 ± 11.5 22.4 ± 11.1 .117 
Hemoglobin, g/L, mean ± SD (n = 352)* 109 ± 24 118 ± 28 .002 107 ± 22 118 ± 14 .171 
Platelet, × 106/L, mean ± SD (n = 355)* 447.0 ± 403.2 408.7 ± 308.0 .840 392.8 ± 255.0 422.5 ± 278.6 .764 
Peripheral blast cells, %, mean ± SD (n = 240)* 1.0 ± 2.6 0.5 ± 1.7 .090 1.4 ± 2.4 1.8 ± 1.8 .299 
Constitutional symptoms, no. (%) (n = 257)* 26 (28.3) 41 (28.1) .976 3 (27.3) 5 (62.5) .125 
Splenomegaly, no. (%) (n = 352)*   .747   .518 
    0 41 (29.7) 50 (26.0)  1 (7.7) 0 (0.0)  
    1 58 (42.0) 83 (43.2)  6 (46.2) 3 (33.3)  
    2 39 (28.3) 59 (30.7)  6 (46.2) 6 (66.7)  
Unfavorable karyotype, no. (%) (n = 137) 8 (12.1) 10 (16.7) .466 2 (28.6) 1 (25.0) .898 
JAK2V617F allele burden, %, mean ± SD  45.9 ± 23.0   42.2 ± 22.1  
MPL W515L/K, no. (%) (n = 354)* 12 (8.8) 1 (0.5) < .0001 0 (0.0) 0 (0.0)  
IWG-MRT score, no. (%) (n = 192)*   .484   .247 
    Low 26 (36.6) 29 (28.4) 3 (27.3) 1 (12.5) 
    Int-1 14 (19.7) 30 (29.4) 4 (36.4) 1 (12.5) 
    Int-2 18 (25.4) 25 (24.5) 0 (0.0) 0 (0.0) 
    High 13 (18.3) 18 (17.6) 4 (36.4) 6 (75.0) 
Progression to acute leukemia, no. (%) (n = 345)* 26 (19.3) 31 (16.5) .520 6 (46.2) 1 (11.1) .083 
Death, no. (%) (n = 352)* 38 (27.7) 48 (24.7) .541 7 (53.8) 6 (75.0) .332 

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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