Under the auspices of an International Working Group, seven centers submitted diagnostic and follow-up information on 1545 patients with World Health Organization-defined polycythemia vera (PV). At diagnosis, median age was 61 years (51% females); thrombocytosis and venous thrombosis were more frequent in women and arterial thrombosis and abnormal karyotype in men. Considering patients from the center with the most mature follow-up information (n=337 with 44% of patients followed to death), median survival (14.1 years) was significantly worse than that of the age- and sex-matched US population (P<0.001). In multivariable analysis, survival for the entire study cohort (n=1545) was adversely affected by older age, leukocytosis, venous thrombosis and abnormal karyotype; a prognostic model that included the first three parameters delineated risk groups with median survivals of 10.9-27.8 years (hazard ratio (HR), 10.7; 95% confidence interval (CI): 7.7-15.0). Pruritus was identified as a favorable risk factor for survival. Cumulative hazard of leukemic transformation, with death as a competing risk, was 2.3% at 10 years and 5.5% at 15 years; risk factors included older age, abnormal karyotype and leukocytes >/=15 x 10(9)/l. Leukemic transformation was associated with treatment exposure to pipobroman or P32/chlorambucil. We found no association between leukemic transformation and hydroxyurea or busulfan use.

Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study / Tefferi, A; Rumi, E; Finazzi, G; Gisslinger, H; Vannucchi, Am; Rodeghiero, F; Randi, Ml; Vaidya, R; Cazzola, M; Rambaldi, A; Gisslinger, B; Pieri, L; Ruggeri, M; Bertozzi, I; Sulai, Nh; Casetti, I; Carobbio, A; Jeryczynski, G; Larson, Dr; Mullauer, L; Pardanani, A; Thiele, J; Passamonti, F; Barbui, T. - In: LEUKEMIA. - ISSN 1476-5551. - 27:9(2013), pp. 1874-1881. [10.1038/leu.2013.163]

Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study

Carobbio A;
2013

Abstract

Under the auspices of an International Working Group, seven centers submitted diagnostic and follow-up information on 1545 patients with World Health Organization-defined polycythemia vera (PV). At diagnosis, median age was 61 years (51% females); thrombocytosis and venous thrombosis were more frequent in women and arterial thrombosis and abnormal karyotype in men. Considering patients from the center with the most mature follow-up information (n=337 with 44% of patients followed to death), median survival (14.1 years) was significantly worse than that of the age- and sex-matched US population (P<0.001). In multivariable analysis, survival for the entire study cohort (n=1545) was adversely affected by older age, leukocytosis, venous thrombosis and abnormal karyotype; a prognostic model that included the first three parameters delineated risk groups with median survivals of 10.9-27.8 years (hazard ratio (HR), 10.7; 95% confidence interval (CI): 7.7-15.0). Pruritus was identified as a favorable risk factor for survival. Cumulative hazard of leukemic transformation, with death as a competing risk, was 2.3% at 10 years and 5.5% at 15 years; risk factors included older age, abnormal karyotype and leukocytes >/=15 x 10(9)/l. Leukemic transformation was associated with treatment exposure to pipobroman or P32/chlorambucil. We found no association between leukemic transformation and hydroxyurea or busulfan use.
2013
27
9
1874
1881
Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study / Tefferi, A; Rumi, E; Finazzi, G; Gisslinger, H; Vannucchi, Am; Rodeghiero, F; Randi, Ml; Vaidya, R; Cazzola, M; Rambaldi, A; Gisslinger, B; Pieri, L; Ruggeri, M; Bertozzi, I; Sulai, Nh; Casetti, I; Carobbio, A; Jeryczynski, G; Larson, Dr; Mullauer, L; Pardanani, A; Thiele, J; Passamonti, F; Barbui, T. - In: LEUKEMIA. - ISSN 1476-5551. - 27:9(2013), pp. 1874-1881. [10.1038/leu.2013.163]
Tefferi, A; Rumi, E; Finazzi, G; Gisslinger, H; Vannucchi, Am; Rodeghiero, F; Randi, Ml; Vaidya, R; Cazzola, M; Rambaldi, A; Gisslinger, B; Pieri, L; Ruggeri, M; Bertozzi, I; Sulai, Nh; Casetti, I; Carobbio, A; Jeryczynski, G; Larson, Dr; Mullauer, L; Pardanani, A; Thiele, J; Passamonti, F; Barbui, T
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11380/1331618
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