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

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 × 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.

• Authors: A Tefferi, E Rumi, G Finazzi, H Gisslinger, A M Vannucchi, F Rodeghiero, M L Randi, R Vaidya, M Cazzola, A Rambaldi, B Gisslinger, L Pieri, M Ruggeri, I Bertozzi, N H Sulai, I Casetti, A Carobbio, G Jeryczynski, D R Larson, L Müllauer, A Pardanani, J Thiele, F Passamonti, T Barbui
• Journal: Leukemia (Leukemia) Vol. 27 Issue 9 Pg. 1874-81 (Sep 2013) ISSN: 1476-5551 [Electronic] England
• PMID: 23739289 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Topics

• Adolescent
• Adult
• Age Factors
• Aged
• Aged, 80 and over
• Cell Transformation, Neoplastic
• Cohort Studies
• Disease Progression
• Female
• Humans
• Incidence
• Leukemia (epidemiology)
• Male
• Middle Aged
• Polycythemia Vera (diagnosis, mortality, therapy)
• Prognosis
• ROC Curve
• Young Adult