Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia.
OBJECTIVE
To assess life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia.


METHODS
The study sample consisted of 831 consecutive patients with polycythemia vera (n = 396; 4184 person-years of follow-up) or essential thrombocythemia (n = 435; 4304 person-years of follow-up). Mortality in each group was compared with the Italian population using the standardized mortality ratio (SMR) based on life expectancy data obtained from the Italian Institute of Statistics.


RESULTS
The 15-year survival was 65% in patients with polycythemia and 73% in those with thrombocythemia. By Cox regression analysis, the independent predictors of death were a history of thrombosis for polycythemia (hazard ratio [HR] = 2.2; P = 0.0002) and thrombocythemia (HR = 2; P = 0.01), and male sex (HR = 1.8; P = 0.03) for thrombocythemia. Mortality compared with the general population was 1.6-fold higher (P <0.001) in patients with polycythemia but was not increased in those with thrombocythemia (SMR = 1; P = 0.8).


CONCLUSIONS
Life expectancy of patients with polycythemia vera (especially if younger than 50 years) was reduced compared with the general population, whereas life expectancy of patients with essential thrombocythemia was not affected significantly by the disease, reflecting the more indolent nature of the proliferation. History of thrombosis was the main predictor of death in both diseases.
Passamonti
Francesco
F
Division of Hematology, IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy. f.passamonti@smatteo.pv.it
Rumi
Elisa
E
Pungolino
Ester
E
Malabarba
Lucia
L
Bertazzoni
Paola
P
Valentini
Marina
M
Orlandi
Ester
E
Arcaini
Luca
L
Brusamolino
Ercole
E
Pascutto
Cristiana
C
Cazzola
Mario
M
Morra
Enrica
E
Lazzarino
Mario
M
D005260:Female; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008017:Life Expectancy; D008297:Male; D008875:Middle Aged; D011087:Polycythemia Vera / Q000150:complications / Q000401:mortality*; D011379:Prognosis; D016016:Proportional Hazards Models; D012307:Risk Factors; D016019:Survival Analysis; D013920:Thrombocythemia, Essential / Q000150:complications / Q000401:mortality*