Treatment outcome in a cohort of young patients with polycythemia vera

The treatment of polycythemia vera in young adults is challenging, requiring one to run a balance between the increased risk of vascular complications, if left untreated, versus the potential of promoting secondary leukemia/myelodysplasia or cancer, if actively managed with chemotherapy. We report the results of a 20-year retrospective analysis in a cohort of 30 young adults with PV (median age 37 years, range 19-45) treated exclusively with phlebotomy, aspirin and hydroxyurea only in case of vascular complications occurring in the presence of thrombocytosis (platelet count > 600 x 10(9)/L). With this approach, vascular complications were no higher than in other published series, and secondary leukemia/myelodisplasia or cancer was not observed during a follow-up of 14 years.