Supplemental table 1: Presenting clinical and laboratory features of 755 Italian patients with

essential thrombocythemia, polycythemia vera or primary myelofibrosis

Variables ET PV PMF P P P

(n=284) (n=310) (n=161) ET ET PV

Vs Vs Vs

PV PMF PMF

Age in years; median (range) 54 (12-88) 58 (19-89) 63 (17-90) 0.001 <0.0001 0.0003

Age ≥60 years; n (%) 107 (37.7%) 141 (45.5%) 99 (61.5%) 0.05 <0.0001 0.005

Females (%) 194 (68.3%) 142 (45.8%) 52 (32.3%) <0.0001 <0.0001 0.001

Hemoglobin, g/dL; median (range) 14.1 (11.0-17.4) 18.0 (13.6-23.2) 11.4 (5.2-17.5) <0.0001 <0.0001 <0.0001

Leukocytes, x 109/L; median (range) 9.0 (2.6-18.2) 10.3 (5.1-25.0) 10.0 (1.4-106.1) <0.0001 0.006 0.8

Platelets, x 109/L; median (range) 766 (469-2348) 459 (159-1639) 285 (19-1563) <0.0001 <0.0001 <0.0001

Leukocytes ≥11 x 109/L; % 22.3% 42.9% 47.8% <0.0001 <0.0001 0.6

Platelets >1,000 x 109/L; % 20.1% 2.1% 5.9% <0.0001 0.0002 0.1

Mutational status, n (%) NA 0.4 NA

JAK2 179 (63%) 310 (100%) 109 (67.7%)

CALR 45 (15.8%) 28 (17.4%)

MPL 14 (5%) 5 (3.1%)

Triple-negative 46(16.2%) 19 (11.8%)

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Supplemental table 2: Presenting clinical and laboratory features of 573 Mayo Clinic patients with

polycythemia vera (n=267) versus JAK2-mutated essential thrombocythemia (n=157) versus JAK2-

mutated primary myelofibrosis (n=149)

Variables ET PV PMF P P P

(n=157) (n=267) (n=149) ET vs. ET vs. PV vs.

PV PMF PMF
Age in years; median (range) 59 (16-88) 64 (19-95) 65 (38-87) 0.03 <0.0001 0.04
Age ≥60 years; n (%) 76 (48.4%) 157 (58.8%) 103 (69.1%) 0.04 0.0002 0.04
Females; n (%) 101 (64.3%) 137 (51.3%) 55 (36.9%) 0.009 <0.0001 0.0047
Hemoglobin, g/dL; median (range) 14.2 (9.8-17.9) 18.4 (15.1-24.5) 10.2 (6.8-16.1) <0.0001 <0.0001 <0.0001
Leukocytes, x 109/L; median (range) 10.0 (3.9-53.4) 11.8 (3.8-171.6) 9.9 (0.8-99.2) <0.0001 0.6 0.002
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Platelets, x 10 /L; median (range) 960 (500-3000) 467 (37-1720) 240 (12-2466) <0.0001 <0.0001 <0.0001
Risk stratification*

Low 30.3% 25.5% 13%

Intermediate 42%

Intermediate-1 10.1% 20.5%

Intermediate-2 21.7% 35.7%

High 27.7% 42.7% 30.8%

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Leukocytes ≥11 x 10 /L; n (%) 39% 56.5% 44.3% 0.0005 0.3 0.02
Platelets >1,000 x 109/L; n (%) 43.9% 7.1% 2% <0.0001 <0.0001 0.03
Abnormal karyotype, n (%) 11 (9%) 26 (18.3%) 55 (37.9%) 0.03 <0.0001 0.0002

” N” evaluable = 409
Palpable splenomegaly, n (%) 22.9% 37.3% 75.2% 0.002 <0.0001 <0.0001
Microcirculatory symptoms, n (%) 32 (20.4%) 89 (40.8%) NA <0.0001 NA NA

“N” evaluable=375

*Risk stratification in primary myelofibrosis (PMF) was according to the dynamic international prognostic scoring system (DIPSS)-plus; in

essential thrombocythemia (ET) according to the international prognostic scoring system for ET (IPSET) and in polycythemia vera (PV)

according to the international working group for myeloproliferative neoplasms research and treatment (IWG-MRT) criteria. References for these

prognostic criteria are included in the main text.

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Supplemental figure 1: Comparison of survival in 389 young (age <60 years) Mayo Clinic patients

with essential thrombocythemia versus polycythemia vera versus primary myelofibrosis. Survival in

essential thrombocythemia was also compared to the age- and sex-matched US population

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Supplemental figure 2: Comparison of survival in 755 Italian patients with essential thrombocythemia

(ET) versus polycythemia vera (PV) versus primary myelofibrosis (PMF).