%0 Journal Article %A Alvarez-Larrán, Alberto %A Kerguelen, Ana %A Hernández-Boluda, Juan C %A Pérez-Encinas, Manuel %A Ferrer-Marín, Francisca %A Bárez, Abelardo %A Martínez-López, Joaquín %A Cuevas, Beatriz %A Mata, M Isabel %A García-Gutiérrez, Valentín %A Aragües, Pilar %A Montesdeoca, Sara %A Burgaleta, Carmen %A Caballero, Gonzalo %A Hernández-Rivas, J Angel %A Durán, M Antonia %A Gómez-Casares, M Teresa %A Besses, Carles %A Grupo Español de Enfermedades Mieloproliferativas Filadelfia Negativas (GEMFIN) %T Frequency and prognostic value of resistance/intolerance to hydroxycarbamide in 890 patients with polycythaemia vera. %D 2015 %U https://hdl.handle.net/10668/26696 %X The clinical significance of resistance/intolerance to hydroxycarbamide (HC) was assessed in a series of 890 patients with polycythaemia vera (PV). Resistance/intolerance to HC was recorded in 137 patients (15·4%), consisting of: need for phlebotomies (3·3%), uncontrolled myeloproliferation (1·6%), failure to reduce massive splenomegaly (0·8%), development of cytopenia at the lowest dose of HC to achieve a response (1·7%) and extra-haematological toxicity (9%). With a median follow-up of 4·6 years, 99 patients died, resulting in a median survival of 19 years. Fulfilling any of the resistance/intolerance criteria had no impact on survival but when the different criteria were individually assessed, an increased risk of death was observed in patients developing cytopenia [Hazard ratio (HR): 3·5, 95% confidence interval (CI): 1·5-8·3, P = 0·003]. Resistance/intolerance had no impact in the rate of thrombosis or bleeding. Risk of myelofibrotic transformation was significantly higher in those patients developing cytopenia (HR: 5·1, 95% CI: 1·9-13·7, P = 0·001) and massive splenomegaly (HR: 9·1, 95% CI: 2·3-35·9, P = 0·002). Cytopenia at the lowest dose required to achieve a response was also an independent risk factor for transformation to acute leukaemia (HR: 20·3, 95% CI: 5·4-76·5, P %K hydroxycarbamide %K myeloproliferative neoplasms %K polycythaemia vera %K resistance/intolerance %K response criteria %~