RESUMEN
Three hundred and twenty-seven patients from two population-based cohorts with an established diagnosis of polycythaemia vera were studied for prognostic risk factors for survival and leukaemia in a long-term survey. The relative survival (RS) was 72% and 46% at 10 and 20 years respectively, from the time of diagnosis. Multivariate analysis identified age >70 years, white blood cell count >13 × 10(9) /l and thrombo-embolism at diagnosis as independent risk factors. Patients with two or three of these factors had a 10 year RS of 26%, compared with 59% and 84% in patients with one and no risk factors, respectively. Age and leucocyte count are the main predicting factors for survival in polycythaemia vera.
Asunto(s)
Leucocitosis/mortalidad , Policitemia Vera/mortalidad , Trombosis/mortalidad , Adulto , Anciano , Anciano de 80 o más Años , Causas de Muerte , Comorbilidad , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Francia/epidemiología , Insuficiencia Cardíaca/mortalidad , Humanos , Estimación de Kaplan-Meier , Leucemia Mieloide Aguda/mortalidad , Recuento de Leucocitos , Leucocitosis/etiología , Masculino , Persona de Mediana Edad , Neoplasias/mortalidad , Policitemia Vera/sangre , Pronóstico , Modelos de Riesgos Proporcionales , Factores de Riesgo , Suecia/epidemiología , Trombosis/etiología , Adulto JovenRESUMEN
To observe the effect of the new World Health Organization (WHO) criteria on the incidence of myeloproliferative neoplasms, we performed a retrospective study of a population-based registry in the Côte d'Or area, France, from 1980 to 2007. A total of 524 myeloproliferative neoplasms were registered for the 1980-2007 period, including 135 polycythemia vera, 308 essential thrombocythemia and 81 idiopathic myelofibroses. No change in the incidence of either polycythemia vera or idiopathic myelofibrosis was observed for the 2005-2007 period, compared to 1980-2004. On the contrary, a pronounced increase in the incidence of essential thrombocythemia was noted after 2005, mainly due to the use of JAK2 mutation screening and a lower threshold of platelet count. Our study confirms the relevance of the new WHO diagnostic criteria in allowing earlier diagnosis of essential thrombocythemia.