Supplemental table 1: Center-stratified patient demographics, median observation periods,
deaths and leukemic transformations among 1,545 patients with World Health Organizationdefined polycythemia vera
Center
Pavia
N
175
364 (48%)
57 years
(18-84)
53/123
(15%/34%)
5.5 years
(0-27)
23
(6%)
15
(4%)
163
(48%)
64 years
(19-95)
34/69
(10%/21%)
7.9 years
(0-39)
147
(44%)
17
(5%)
113
(51%)
94
(44%)
82
(54%)
81
(56%)
52
(48%)
60 years
(20-88)
63 years
(21-92)
62 years
(28-91)
59 years
(20-89)
64 years
(22-85)
19/55
(9%/25%)
16/49
(8%/23%)
8/24
(5%/16%)
10/32
(7%/22%)
14/24
(13%/22%)
7.9 years
(0-29)
7.7 years
(0-33.3)
4.5 years
(0-19)
8.5 years
(1-31)
7.8 years
(0-30)
58
(26%)
75
(35%)
8
(5%)
19
(13%)
17
(16%)
8
(4%)
5
(2%)
2
(1%)
2
(1%)
1
(1%)
1,545 760
(49%)
61 years
(18-95)
347
154/376
6.9 years
(23%)
(10%/24%) (0-39)
50
(3%)
Rochester 337
Bergamo
223
Vienna
213
Florence
153
Vicenza
146
Padua
109
Total
Median age Age below Median
Leukemic
Males at diagnosis 40/50 years follow-up Deaths transformations
n (%)
(range)
n (%)
(range) n (%)
n (%)
1
Supplemental table 2: Overall survival prediction among 1,545 patients with polycythemia vera
using cutoff values for age and leukocyte count, as determined by receiver operating
characteristic analysis (age cutoff = <57, 57-66 and ≥67 years; leukocyte cutoff = 15 x 109/L)
Overall survival
Age categories (years)
≥67
57-66
Leukocyte count ≥15 x 109/L
Thrombocytosis ( ≥450 x 109/L)
Extreme thrombocytosis
( ≥1,000 x 109/L)
Arterial thrombosis
(at or before diagnosis)
Venous thrombosis
(at or before diagnosis)
Hypertension
Diabetes
Leukoerythroblastic blood smear
Pruritus
Abnormal karyotype
N
evaluable
Univariate
P values
Age-adjusted
P values
1,545
<0.0001
1,545
<0.0001
<0.0001
1,545
1,545
0.003
0.04
0.02
0.26
1,545
0.0007
0.21
1,545
0.008
0.001
1,388
1,149
1,056
1,349
<0.0001
0.003
0.002
0.04
0.25
0.10
0.002
0.008
631
<0.0001
<0.0001
Multivariable
P values*
(HR ; 95% CI)
N=964
Multivariable
P values**
(HR ; 95% CI)
N=383
<0.0001
(8.5; 5.7-12.6)
(2.9; 1.9-4.4)
<0.0001
(2.2; 1.6-3.0)
0.1
<0.0001
(12.1; 5.6-26.4)
(4.0; 1.7-9.0)
<0.0001
(4.1; 2.4-6.9)
0.47
0.001
(1.8; 1.1-2.8)
0.0004
(3.7; 1.8-7.6)
0.06
0.005
(0.7; 0.5-0.9)
0.94
0.02
(0.5; 0.3-0.9)
0.0009
(3.0; 1.6-5.6)
*Karyotype excluded as a covariate
**Karyotype included as a covariate
2
Legend:
Supplemental figure 1: Overall survival in 1,545 patients with polycythemia vera stratified by center of
origin.
Supplemental figure 2: Survival in 337 Mayo Clinic patients with polycythemia vera, stratified by age
group (<57, 57-66, and 67+ years), and compared to expected survival based on individuals of the same
age and gender from the US Total population; the respective number of patients in each age category was
117, 90 and 130.
Supplemental figure 3: Karyotype-inclusive risk-stratified survival in 631 patients with polycythemia
vera. The presence of abnormal karyotype increased the risk level to the next higher level: best survival
curve = low risk with normal karyotype; next best curve = low risk with abnormal karyotype or
intermediate risk with normal karyotype; third best curve = intermediate risk with abnormal karyotype or
high risk with normal karyotype; worst survival curve = high risk with abnormal karyotype.
Supplemental figure 4: Cumulative probability of leukemic transformation in 1,545 patients with
polycythemia vera, stratified by treatment exposure history, as outlined in table 2.
3
4
5
6
7