Long-term follow-up of 386 consecutive patients with essential thrombocythemia: Safety of cytoreductive therapy (pages 215–220)

摘要

Cytotoxic agents like Hydroxyurea, Busulfan and Interferon-alpha are to date the most commonly used therapeuticnapproaches in Essential Thrombocythemia (ET). However, few data on the efficacy and safety ofnthese agents in the long-term are currently available. We report a retrospective analysis of the long-termnoutcome of 386 consecutive ET patients, followed at single Institution for a median follow-up of 9.5 yearsn(range, 3–28.5). Cytoreductive therapy was administered to 338 patients (88%), obtaining a response in 86%nof cases. Forty-five patients (12%) experienced a thrombosis. Among baseline characteristics, only historynof vascular events prior to ET diagnosis predicted a higher incidence of thrombosis. Evolution in acute leukemiamyelofibrosis occurred in 6 (1,5%) and 20 (5%) patients, and was significantly higher in patients receivingnsequential cytotoxic agents. Overall survival was 38% at 19 years and was poorer for patients older thann60 years, with higher leukocytes count (>15 3 109/L), hypertension and mellitus diabetes at ET diagnosis andnfor patients experiencing a thrombotic event during follow-up. Cytoreductive therapy was effective in decreasingnplatelet number with negligible toxicity; however, thrombocytosis control did not reduce the incidence ofnthrombosis and, for patients who received sequential therapies, the probability of disease evolution wasnhigher and survival was poorer. Am. J. Hematol. 84:215–220, 2009.