Anna Angona1, Alberto Alvarez-Larrán1, Beatriz Bellosillo2, Luz Martínez-Avilés3, Francesc Garcia-Pallarols4, Raquel Longarón5, Àgueda Ancochea1, Carles Besses6. 1. Servicio de Hematología, Hospital del Mar-IMIM, Barcelona, España; Universitat Autònoma de Barcelona, Barcelona, España; Grup de Recerca Clínica Aplicada en Neoplàsies Hematològiques, Hospital del Mar-IMIM, Barcelona, España. 2. Grup de Recerca Clínica Aplicada en Neoplàsies Hematològiques, Hospital del Mar-IMIM, Barcelona, España; Servicio de Patología, Hospital del Mar-IMIM, Barcelona, España; Universitat Pompeu Fabra, Barcelona, España. 3. Universitat Autònoma de Barcelona, Barcelona, España; Grup de Recerca Clínica Aplicada en Neoplàsies Hematològiques, Hospital del Mar-IMIM, Barcelona, España; Servicio de Patología, Hospital del Mar-IMIM, Barcelona, España. 4. Servicio de Hematología, Hospital del Mar-IMIM, Barcelona, España; Grup de Recerca Clínica Aplicada en Neoplàsies Hematològiques, Hospital del Mar-IMIM, Barcelona, España. 5. Grup de Recerca Clínica Aplicada en Neoplàsies Hematològiques, Hospital del Mar-IMIM, Barcelona, España; Servicio de Patología, Hospital del Mar-IMIM, Barcelona, España. 6. Servicio de Hematología, Hospital del Mar-IMIM, Barcelona, España; Grup de Recerca Clínica Aplicada en Neoplàsies Hematològiques, Hospital del Mar-IMIM, Barcelona, España. Electronic address: cbesses@parcdesalutmar.cat.
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 center 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>10×10(9)/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>10×10(9)/l were significantly associated with lower TFS, while the prognostic IPSET-Thrombosis system did not provide more information than classical thrombotic risk assessment.
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 center 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>10×10(9)/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>10×10(9)/l were significantly associated with lower TFS, while the prognostic IPSET-Thrombosis system did not provide more information than classical thrombotic risk assessment.