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

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. MüllauerA. Pardanani, J. Thiele, F. Passamonti, T. Barbui

Research output: Contribution to journalArticlepeer-review

333 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)1874-1881
Number of pages8
Issue number9
StatePublished - Sep 2013


  • myelofibrosis
  • polycythemia
  • prognosis
  • survival
  • thrombocythemia

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research


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