A lower intensity of treatment may underlie the increased risk of thrombosis in young patients with masked polycythaemia vera

Federico Lussana, Alessandra Carobbio, Maria L. Randi, Chiara Elena, Elisa Rumi, Guido Finazzi, Irene Bertozzi, Lisa Pieri, Marco Ruggeri, Francesca Palandri, Nicola Polverelli, Elena Elli, Alessia Tieghi, Alessandra Iurlo, Marco Ruella, Mario Cazzola, Alessandro Rambaldi, Alessandro M. Vannucchi, Tiziano Barbui

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

Summary: In patients who do not meet the World Health Organization (WHO) criteria for overt polycythaemia vera (PV), a diagnosis of masked PV (mPV) can be determined. A fraction of mPV patients may display thrombocytosis, thus mimicking essential thrombocythaemia (ET). No previous studies have examined clinical outcomes of mPV among young JAK2-mutated patients. We analysed a retrospective cohort of 538 JAK2-mutated patients younger than 40 years, after a re-assessment of the diagnosis according to the haemoglobin threshold for mPV. In this cohort of patients, 97 (18%) met the WHO criteria for PV, 66 patients (12%) were classified as mPV and 375 (70%) as JAK2-mutated ET. Surprisingly, a significant difference in the incidence of thrombosis was found when comparing mPV versus overt PV patients (P = 0·04). In multivariate analysis, the only factor accounting for the difference in the risk of thrombosis was the less frequent use of phlebotomies and cytoreduction in mPV patients compared to those with overt PV. Thus, we emphasize the need for the identification of mPV in young JAK2-mutated patients in order to optimize their treatments.

Original languageEnglish
Pages (from-to)541-546
Number of pages6
JournalBritish Journal of Haematology
Volume167
Issue number4
DOIs
Publication statusPublished - Nov 1 2014

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Polycythemia Vera
Thrombosis
Essential Thrombocythemia
Therapeutics
Thrombocytosis
Phlebotomy
Hemoglobins
Multivariate Analysis

Keywords

  • Essential thrombocythaemia
  • JAK2 mutation
  • Masked polycythaemia
  • Polycythaemia vera
  • Thrombosis

ASJC Scopus subject areas

  • Hematology
  • Medicine(all)

Cite this

A lower intensity of treatment may underlie the increased risk of thrombosis in young patients with masked polycythaemia vera. / Lussana, Federico; Carobbio, Alessandra; Randi, Maria L.; Elena, Chiara; Rumi, Elisa; Finazzi, Guido; Bertozzi, Irene; Pieri, Lisa; Ruggeri, Marco; Palandri, Francesca; Polverelli, Nicola; Elli, Elena; Tieghi, Alessia; Iurlo, Alessandra; Ruella, Marco; Cazzola, Mario; Rambaldi, Alessandro; Vannucchi, Alessandro M.; Barbui, Tiziano.

In: British Journal of Haematology, Vol. 167, No. 4, 01.11.2014, p. 541-546.

Research output: Contribution to journalArticle

Lussana, F, Carobbio, A, Randi, ML, Elena, C, Rumi, E, Finazzi, G, Bertozzi, I, Pieri, L, Ruggeri, M, Palandri, F, Polverelli, N, Elli, E, Tieghi, A, Iurlo, A, Ruella, M, Cazzola, M, Rambaldi, A, Vannucchi, AM & Barbui, T 2014, 'A lower intensity of treatment may underlie the increased risk of thrombosis in young patients with masked polycythaemia vera', British Journal of Haematology, vol. 167, no. 4, pp. 541-546. https://doi.org/10.1111/bjh.13080
Lussana, Federico ; Carobbio, Alessandra ; Randi, Maria L. ; Elena, Chiara ; Rumi, Elisa ; Finazzi, Guido ; Bertozzi, Irene ; Pieri, Lisa ; Ruggeri, Marco ; Palandri, Francesca ; Polverelli, Nicola ; Elli, Elena ; Tieghi, Alessia ; Iurlo, Alessandra ; Ruella, Marco ; Cazzola, Mario ; Rambaldi, Alessandro ; Vannucchi, Alessandro M. ; Barbui, Tiziano. / A lower intensity of treatment may underlie the increased risk of thrombosis in young patients with masked polycythaemia vera. In: British Journal of Haematology. 2014 ; Vol. 167, No. 4. pp. 541-546.
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AU - Lussana, Federico

AU - Carobbio, Alessandra

AU - Randi, Maria L.

AU - Elena, Chiara

AU - Rumi, Elisa

AU - Finazzi, Guido

AU - Bertozzi, Irene

AU - Pieri, Lisa

AU - Ruggeri, Marco

AU - Palandri, Francesca

AU - Polverelli, Nicola

AU - Elli, Elena

AU - Tieghi, Alessia

AU - Iurlo, Alessandra

AU - Ruella, Marco

AU - Cazzola, Mario

AU - Rambaldi, Alessandro

AU - Vannucchi, Alessandro M.

AU - Barbui, Tiziano

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N2 - Summary: In patients who do not meet the World Health Organization (WHO) criteria for overt polycythaemia vera (PV), a diagnosis of masked PV (mPV) can be determined. A fraction of mPV patients may display thrombocytosis, thus mimicking essential thrombocythaemia (ET). No previous studies have examined clinical outcomes of mPV among young JAK2-mutated patients. We analysed a retrospective cohort of 538 JAK2-mutated patients younger than 40 years, after a re-assessment of the diagnosis according to the haemoglobin threshold for mPV. In this cohort of patients, 97 (18%) met the WHO criteria for PV, 66 patients (12%) were classified as mPV and 375 (70%) as JAK2-mutated ET. Surprisingly, a significant difference in the incidence of thrombosis was found when comparing mPV versus overt PV patients (P = 0·04). In multivariate analysis, the only factor accounting for the difference in the risk of thrombosis was the less frequent use of phlebotomies and cytoreduction in mPV patients compared to those with overt PV. Thus, we emphasize the need for the identification of mPV in young JAK2-mutated patients in order to optimize their treatments.

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