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Inicio Medicina Clínica (English Edition) Essential thrombocythemia: Baseline characteristics and risk factors for surviva...
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Vol. 144. Issue 6.
Pages 247-253 (March 2015)
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Vol. 144. Issue 6.
Pages 247-253 (March 2015)
Original article
DOI: 10.1016/j.medcle.2015.10.002
Essential thrombocythemia: Baseline characteristics and risk factors for survival and thrombosis in a series of 214 patients
Trombocitemia esencial: características iniciales y factores de riesgo de supervivencia y trombosis en una serie de 214 pacientes
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Anna Angonaa,b,c, Alberto Alvarez-Larrána,b,c, Beatriz Bellosilloc,d,e, Luz Martínez-Avilésb,c,d, Francesc Garcia-Pallarolsa,c, Raquel Longarónc,d, Àgueda Ancocheaa,b,c, Carles Bessesa,c,
Corresponding author
cbesses@parcdesalutmar.cat

Corresponding author.
a Servicio de Hematología, Hospital del Mar-IMIM, Barcelona, Spain
b Universitat Autònoma de Barcelona, Barcelona, Spain
c Grup de Recerca Clínica Aplicada en Neoplàsies Hematològiques, Hospital del Mar-IMIM, Barcelona, Spain
d Servicio de Patología, Hospital del Mar-IMIM, Barcelona, Spain
e Universitat Pompeu Fabra, Barcelona, Spain
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Tables (6)
Table 1. Main clinical-biological characteristics of the 214 patients with essential thrombocythaemia at the moment of diagnosis.
Table 2a. Main clinical-biological characteristics at the moment of diagnosis of 204 patients with essential thrombocythaemia based on the mutation state of JAK2V617F.
Table 2b. Main clinical-biological characteristics at the moment of diagnosis of 159 patients with essential thrombocythaemia based on the mutation presence of JAK2V617F and calreticulin mutations.
Table 3. Factors associated with overall survival in the univariate analysis of 214 patients with essential thrombocythaemia.
Table 4. Type and number of major thrombotic episodes after diagnosis in 214 patients with essential thrombocythaemia.
Table 5. Risk factors associated with thrombosis-free survival in the univariate analysis of 214 patients with essential thrombocythaemia.
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Abstract
Background and objective

Two prognostic models to predict overall survival and thrombosis-free survival have been proposed: International Prognostic Score for Essential Thrombocythemia (IPSET) and IPSET-Thrombosis, respectively, based on age, leukocytes count, history of previous thrombosis, the presence of cardiovascular risk factors and the JAK2 mutational status. The aim of the present study was to assess the clinical and biological characteristics at diagnosis and during evolution in essential thrombocythemia (ET) patients as well as the factors associated with survival and thrombosis and the usefulness of these new prognostic models.

Patients and methods

We have evaluated the clinical data and the mutation status of JAK2, MPL and calreticulin of 214 ET patients diagnosed in a single centre between 1985 and 2012, classified according to classical risk stratification, IPSET and IPSET-Thrombosis.

Results

With a median follow-up of 6.9 years, overall survival was not associated with any variable by multivariate analysis. Thrombotic history and leukocytes >10×109/L were associated with thrombosis-free survival (TFS). In our series, IPSET prognostic systems of survival and thrombosis did not provide more clinically relevant information regarding the classic risk of thrombosis stratification.

Conclusion

Thrombotic history and leukocytosis >10×109/L were significantly associated with lower TFS, while the prognostic IPSET-Thrombosis system did not provide more information than classical thrombotic risk assessment.

Keywords:
Essential thrombocythemia
Thrombosis
Survival
JAK2V617F
Calreticulin
Resumen
Introducción y objetivo

Recientemente se han publicado 2 nuevos índices pronósticos de supervivencia y de trombosis, el International Prognostic Score for Essential Thrombocythemia (IPSET) y el IPSET-Thrombosis, respectivamente, basados en la edad, la cifra de leucocitos, la historia de trombosis, la presencia de factores de riesgo cardiovascular y el estado mutacional de JAK2. El objetivo del presente estudio fue analizar las características clínico-biológicas en el momento del diagnóstico y durante la evolución en una serie homogénea de pacientes con trombocitemia esencial (TE), así como analizar los factores asociados a la supervivencia y a la trombosis y la utilidad de dichos índices pronósticos.

Pacientes y métodos

Se revisaron los datos analíticos y clínicos y el estado mutacional de JAK2, MPL y calreticulina de 214 pacientes diagnosticados de TE consecutivamente en un único centro entre 1985 y 2012. Se clasificaron los pacientes de acuerdo con la estratificación de riesgo clásica, el IPSET y el IPSET-Thrombosis.

Resultados

Con una mediana de seguimiento de 6,9 años, el análisis multivariado no puso de manifiesto ningún factor asociado a la supervivencia global. Los antecedentes trombóticos y la leucocitosis >10×109/l se asociaron a la supervivencia libre de trombosis (SLT). En nuestra serie, los sistemas pronósticos IPSET de supervivencia y de trombosis no aportan información de mayor relevancia clínica respecto al pronóstico asociado con los factores de riesgo trombótico clásico.

Conclusión

Los antecedentes de trombosis y la leucocitosis >10×109/l fueron las variables asociadas a una SLT inferior, mientras que el sistema pronóstico IPSET-Thrombosis no aportó mayor información que la estratificación clásica de riesgo trombótico.

Palabras clave:
Trombocitemia esencial
Trombosis
Supervivencia
JAK2V617F
Calreticulina

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