Development and validation of an International Prognostic Score of thrombosis in World Health Organization-essential thrombocythemia (IPSET-thrombosis)

Tiziano Barbui, Guido Finazzi, Alessandra Carobbio, Juergen Thiele, Francesco Passamonti, Elisa Rumi, Marco Ruggeri, Francesco Rodeghiero, Maria Luigia Randi, Irene Bertozzi, Heinz Gisslinger, Veronika Buxhofer-Ausch, Valerio De Stefano, Silvia Betti, Alessandro Rambaldi, Alessandro M. Vannucchi, Ayalew Tefferi

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Abstract

Accurate prediction of thrombosis in essential thrombocythemia (ET) provides the platform for prospective studies exploring preventive measures. Current risk stratification for thrombosis in ET is 2-tiered and considers low- and high-risk categories based on the respective absence or presence of either age > 60 years or history of thrombosis. In an international study of 891 patients with World Health Organization (WHO) - defined ET, we identified additional independent risk factors including cardiovascular risk factors and JAK2 V617F. Accordingly, we assigned risk scores based on multivariable analysis-derived hazard ratios (HRs) to age > 60 years (HR = 1.5; 1 point), thrombosis history (HR = 1.9; 2 points), cardiovascular risk factors (HR = 1.6; 1 point), and JAK2 V617F (HR = 2.0; 2 points) and subsequently devised a 3-tiered prognostic model (low-risk = < 2 points; intermediate-risk = 2 points; and high-risk = > 2 points) using a training set of 535 patients and validated the results in the remaining cohort (n = 356; internal validation set) and in an external validation set (n = 329). Considering all 3 cohorts (n = 1220), the 3-tiered new prognostic model (low-risk n = 474 vs intermediate-risk n = 471 vs high-risk n = 275), with a respective thrombosis risk of 1.03% of patients/y versus 2.35% of patients/y versus 3.56% of patients/y, outperformed the 2-tiered (low-risk 0.95% of patients/y vs high-risk 2.86% of patients/y) conventional risk stratification in predicting future vascular events.

Original languageEnglish (US)
Pages (from-to)5128-5133
Number of pages6
JournalBlood
Volume120
Issue number26
DOIs
StatePublished - Dec 20 2012

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Essential Thrombocythemia
Thrombosis
Health
Hazards
Blood Vessels

ASJC Scopus subject areas

  • Hematology
  • Biochemistry
  • Cell Biology
  • Immunology

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Development and validation of an International Prognostic Score of thrombosis in World Health Organization-essential thrombocythemia (IPSET-thrombosis). / Barbui, Tiziano; Finazzi, Guido; Carobbio, Alessandra; Thiele, Juergen; Passamonti, Francesco; Rumi, Elisa; Ruggeri, Marco; Rodeghiero, Francesco; Randi, Maria Luigia; Bertozzi, Irene; Gisslinger, Heinz; Buxhofer-Ausch, Veronika; De Stefano, Valerio; Betti, Silvia; Rambaldi, Alessandro; Vannucchi, Alessandro M.; Tefferi, Ayalew.

In: Blood, Vol. 120, No. 26, 20.12.2012, p. 5128-5133.

Research output: Contribution to journalArticle

Barbui, T, Finazzi, G, Carobbio, A, Thiele, J, Passamonti, F, Rumi, E, Ruggeri, M, Rodeghiero, F, Randi, ML, Bertozzi, I, Gisslinger, H, Buxhofer-Ausch, V, De Stefano, V, Betti, S, Rambaldi, A, Vannucchi, AM & Tefferi, A 2012, 'Development and validation of an International Prognostic Score of thrombosis in World Health Organization-essential thrombocythemia (IPSET-thrombosis)', Blood, vol. 120, no. 26, pp. 5128-5133. https://doi.org/10.1182/blood-2012-07-444067
Barbui, Tiziano ; Finazzi, Guido ; Carobbio, Alessandra ; Thiele, Juergen ; Passamonti, Francesco ; Rumi, Elisa ; Ruggeri, Marco ; Rodeghiero, Francesco ; Randi, Maria Luigia ; Bertozzi, Irene ; Gisslinger, Heinz ; Buxhofer-Ausch, Veronika ; De Stefano, Valerio ; Betti, Silvia ; Rambaldi, Alessandro ; Vannucchi, Alessandro M. ; Tefferi, Ayalew. / Development and validation of an International Prognostic Score of thrombosis in World Health Organization-essential thrombocythemia (IPSET-thrombosis). In: Blood. 2012 ; Vol. 120, No. 26. pp. 5128-5133.
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abstract = "Accurate prediction of thrombosis in essential thrombocythemia (ET) provides the platform for prospective studies exploring preventive measures. Current risk stratification for thrombosis in ET is 2-tiered and considers low- and high-risk categories based on the respective absence or presence of either age > 60 years or history of thrombosis. In an international study of 891 patients with World Health Organization (WHO) - defined ET, we identified additional independent risk factors including cardiovascular risk factors and JAK2 V617F. Accordingly, we assigned risk scores based on multivariable analysis-derived hazard ratios (HRs) to age > 60 years (HR = 1.5; 1 point), thrombosis history (HR = 1.9; 2 points), cardiovascular risk factors (HR = 1.6; 1 point), and JAK2 V617F (HR = 2.0; 2 points) and subsequently devised a 3-tiered prognostic model (low-risk = < 2 points; intermediate-risk = 2 points; and high-risk = > 2 points) using a training set of 535 patients and validated the results in the remaining cohort (n = 356; internal validation set) and in an external validation set (n = 329). Considering all 3 cohorts (n = 1220), the 3-tiered new prognostic model (low-risk n = 474 vs intermediate-risk n = 471 vs high-risk n = 275), with a respective thrombosis risk of 1.03{\%} of patients/y versus 2.35{\%} of patients/y versus 3.56{\%} of patients/y, outperformed the 2-tiered (low-risk 0.95{\%} of patients/y vs high-risk 2.86{\%} of patients/y) conventional risk stratification in predicting future vascular events.",
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AU - Thiele, Juergen

AU - Passamonti, Francesco

AU - Rumi, Elisa

AU - Ruggeri, Marco

AU - Rodeghiero, Francesco

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AU - De Stefano, Valerio

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