A prognostic model to predict survival in 867 World Health Organization - Defined essential thrombocythemia at diagnosis: A study by the International Working Group on Myelofibrosis Research and Treatment

Francesco Passamonti, Jürgen Thiele, Francois Girodon, Elisa Rumi, Alessandra Carobbio, Heinz Gisslinger, Hans Michael Kvasnicka, Marco Ruggeri, Maria Luigia Randi, Naseema Gangat, Alessandro Maria Vannucchi, Andrea Gianatti, Bettina Gisslinger, Leonhard Müllauer, Francesco Rodeghiero, Emanuele S G D'Amore, Irene Bertozzi, Curtis A. Hanson, Emanuela Boveri, Filippo MarinoMargherita Maffioli, Domenica Caramazza, Elisabetta Antonioli, Valentina Carrai, Veronika Buxhofer-Ausch, Cristiana Pascutto, Mario Cazzola, Tiziano Barbui, Ayalew Tefferi

Research output: Contribution to journalArticle

143 Citations (Scopus)

Abstract

Diagnosis of essential thrombocythemia (ET) has been updated in the last World Health Organization (WHO) classification. We developed a prognostic model to predict survival at diagnosis, named IPSET (International Prognostic Score for ET), studying patients with WHO-defined ET. Age 60 years or older, leukocyte count ≥ 11 × 109/L, and prior thrombosis significantly affected survival, by multivariable Cox regression. On the basis of the hazard ratio, we assigned 2 points to age and 1 each to leukocyte count and thrombosis. So, the IPSET model allocated 867 patients into 3 risk categories with significantly different survival: low (sum of points = 0; median survival not reached), intermediate (sum = 1-2; median survival 24.5 years), and high (sum = 3-4, median survival 13.8 years). The IPSET model was further validated in 2 independent cohorts including 132 WHO-defined ET and 234 Polycythemia Vera Study Group-defined ET patients. The IPSET model was able to predict the occurrence of thrombosis, and not to predict post-ET myelofibrosis. In conclusion, IPSET, based on age ≥ 60 years, leukocyte count ≥ 11 × 109/L, and history of thrombosis allows prognostic assessment of WHO-defined ET and the validation process makes IPSET applicable in all patients phenotypically appearing as ET.

Original languageEnglish (US)
Pages (from-to)1197-1201
Number of pages5
JournalBlood
Volume120
Issue number6
DOIs
StatePublished - Aug 9 2012

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Essential Thrombocythemia
Primary Myelofibrosis
Health
Survival
Research
Thrombosis
Leukocyte Count
Therapeutics
Hazards
Polycythemia Vera

ASJC Scopus subject areas

  • Hematology
  • Biochemistry
  • Cell Biology
  • Immunology

Cite this

A prognostic model to predict survival in 867 World Health Organization - Defined essential thrombocythemia at diagnosis : A study by the International Working Group on Myelofibrosis Research and Treatment. / Passamonti, Francesco; Thiele, Jürgen; Girodon, Francois; Rumi, Elisa; Carobbio, Alessandra; Gisslinger, Heinz; Kvasnicka, Hans Michael; Ruggeri, Marco; Randi, Maria Luigia; Gangat, Naseema; Vannucchi, Alessandro Maria; Gianatti, Andrea; Gisslinger, Bettina; Müllauer, Leonhard; Rodeghiero, Francesco; D'Amore, Emanuele S G; Bertozzi, Irene; Hanson, Curtis A.; Boveri, Emanuela; Marino, Filippo; Maffioli, Margherita; Caramazza, Domenica; Antonioli, Elisabetta; Carrai, Valentina; Buxhofer-Ausch, Veronika; Pascutto, Cristiana; Cazzola, Mario; Barbui, Tiziano; Tefferi, Ayalew.

In: Blood, Vol. 120, No. 6, 09.08.2012, p. 1197-1201.

Research output: Contribution to journalArticle

Passamonti, F, Thiele, J, Girodon, F, Rumi, E, Carobbio, A, Gisslinger, H, Kvasnicka, HM, Ruggeri, M, Randi, ML, Gangat, N, Vannucchi, AM, Gianatti, A, Gisslinger, B, Müllauer, L, Rodeghiero, F, D'Amore, ESG, Bertozzi, I, Hanson, CA, Boveri, E, Marino, F, Maffioli, M, Caramazza, D, Antonioli, E, Carrai, V, Buxhofer-Ausch, V, Pascutto, C, Cazzola, M, Barbui, T & Tefferi, A 2012, 'A prognostic model to predict survival in 867 World Health Organization - Defined essential thrombocythemia at diagnosis: A study by the International Working Group on Myelofibrosis Research and Treatment', Blood, vol. 120, no. 6, pp. 1197-1201. https://doi.org/10.1182/blood-2012-01-403279
Passamonti, Francesco ; Thiele, Jürgen ; Girodon, Francois ; Rumi, Elisa ; Carobbio, Alessandra ; Gisslinger, Heinz ; Kvasnicka, Hans Michael ; Ruggeri, Marco ; Randi, Maria Luigia ; Gangat, Naseema ; Vannucchi, Alessandro Maria ; Gianatti, Andrea ; Gisslinger, Bettina ; Müllauer, Leonhard ; Rodeghiero, Francesco ; D'Amore, Emanuele S G ; Bertozzi, Irene ; Hanson, Curtis A. ; Boveri, Emanuela ; Marino, Filippo ; Maffioli, Margherita ; Caramazza, Domenica ; Antonioli, Elisabetta ; Carrai, Valentina ; Buxhofer-Ausch, Veronika ; Pascutto, Cristiana ; Cazzola, Mario ; Barbui, Tiziano ; Tefferi, Ayalew. / A prognostic model to predict survival in 867 World Health Organization - Defined essential thrombocythemia at diagnosis : A study by the International Working Group on Myelofibrosis Research and Treatment. In: Blood. 2012 ; Vol. 120, No. 6. pp. 1197-1201.
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abstract = "Diagnosis of essential thrombocythemia (ET) has been updated in the last World Health Organization (WHO) classification. We developed a prognostic model to predict survival at diagnosis, named IPSET (International Prognostic Score for ET), studying patients with WHO-defined ET. Age 60 years or older, leukocyte count ≥ 11 × 109/L, and prior thrombosis significantly affected survival, by multivariable Cox regression. On the basis of the hazard ratio, we assigned 2 points to age and 1 each to leukocyte count and thrombosis. So, the IPSET model allocated 867 patients into 3 risk categories with significantly different survival: low (sum of points = 0; median survival not reached), intermediate (sum = 1-2; median survival 24.5 years), and high (sum = 3-4, median survival 13.8 years). The IPSET model was further validated in 2 independent cohorts including 132 WHO-defined ET and 234 Polycythemia Vera Study Group-defined ET patients. The IPSET model was able to predict the occurrence of thrombosis, and not to predict post-ET myelofibrosis. In conclusion, IPSET, based on age ≥ 60 years, leukocyte count ≥ 11 × 109/L, and history of thrombosis allows prognostic assessment of WHO-defined ET and the validation process makes IPSET applicable in all patients phenotypically appearing as ET.",
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T1 - A prognostic model to predict survival in 867 World Health Organization - Defined essential thrombocythemia at diagnosis

T2 - A study by the International Working Group on Myelofibrosis Research and Treatment

AU - Passamonti, Francesco

AU - Thiele, Jürgen

AU - Girodon, Francois

AU - Rumi, Elisa

AU - Carobbio, Alessandra

AU - Gisslinger, Heinz

AU - Kvasnicka, Hans Michael

AU - Ruggeri, Marco

AU - Randi, Maria Luigia

AU - Gangat, Naseema

AU - Vannucchi, Alessandro Maria

AU - Gianatti, Andrea

AU - Gisslinger, Bettina

AU - Müllauer, Leonhard

AU - Rodeghiero, Francesco

AU - D'Amore, Emanuele S G

AU - Bertozzi, Irene

AU - Hanson, Curtis A.

AU - Boveri, Emanuela

AU - Marino, Filippo

AU - Maffioli, Margherita

AU - Caramazza, Domenica

AU - Antonioli, Elisabetta

AU - Carrai, Valentina

AU - Buxhofer-Ausch, Veronika

AU - Pascutto, Cristiana

AU - Cazzola, Mario

AU - Barbui, Tiziano

AU - Tefferi, Ayalew

PY - 2012/8/9

Y1 - 2012/8/9

N2 - Diagnosis of essential thrombocythemia (ET) has been updated in the last World Health Organization (WHO) classification. We developed a prognostic model to predict survival at diagnosis, named IPSET (International Prognostic Score for ET), studying patients with WHO-defined ET. Age 60 years or older, leukocyte count ≥ 11 × 109/L, and prior thrombosis significantly affected survival, by multivariable Cox regression. On the basis of the hazard ratio, we assigned 2 points to age and 1 each to leukocyte count and thrombosis. So, the IPSET model allocated 867 patients into 3 risk categories with significantly different survival: low (sum of points = 0; median survival not reached), intermediate (sum = 1-2; median survival 24.5 years), and high (sum = 3-4, median survival 13.8 years). The IPSET model was further validated in 2 independent cohorts including 132 WHO-defined ET and 234 Polycythemia Vera Study Group-defined ET patients. The IPSET model was able to predict the occurrence of thrombosis, and not to predict post-ET myelofibrosis. In conclusion, IPSET, based on age ≥ 60 years, leukocyte count ≥ 11 × 109/L, and history of thrombosis allows prognostic assessment of WHO-defined ET and the validation process makes IPSET applicable in all patients phenotypically appearing as ET.

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