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

2013; Springer Nature; Volume: 27; Issue: 9 Linguagem: Inglês

10.1038/leu.2013.163

1476-5551

Ayalew Tefferi, Elisa Rumi, Guido Finazzi, Heinz Gisslinger, Alessandro M. Vannucchi, Francesco Rodeghiero, Maria Luigia Randi, Rakhee Vaidya, Mario Cazzola, Alessandro Rambaldi, Bettina Gisslinger, Lisa Pieri, Marco Ruggeri, Irene Bertozzi, Nanna Sulai, Ilaria Carola Casetti, Alessandra Carobbio, Georg Jeryczynski, Dirk R. Larson, Leonhard Müllauer, Animesh Pardanani, J. Thiele, Francesco Passamonti, Tiziano Barbui,

Acute Myeloid Leukemia Research

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 × 109/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.