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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244 Scopus citations

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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ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

Barbui, T., Finazzi, G., Carobbio, A., Thiele, J., Passamonti, F., Rumi, E., Ruggeri, M., Rodeghiero, F., Randi, M. L., Bertozzi, I., Gisslinger, H., Buxhofer-Ausch, V., De Stefano, V., Betti, S., Rambaldi, A., Vannucchi, A. M., & Tefferi, A. (2012). Development and validation of an International Prognostic Score of thrombosis in World Health Organization-essential thrombocythemia (IPSET-thrombosis). Blood, 120(26), 5128-5133. https://doi.org/10.1182/blood-2012-07-444067