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CALR mutation, MPL mutation and triple negativity identify patients with the lowest vascular risk in primary myelofibrosis

Leukemia volume 29, pages 1209–1210 (2015)Cite this article

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The thrombotic risk in primary myelofibrosis (PMF) has been underestimated for a long time. In 2010 Barbui et al.1 analyzed retrospectively the frequency and risk factors of major cardiovascular (CV) events in 707 patients with PMF followed-up in 4 European institutions. The overall cumulative rate of CV deaths and nonfatal thrombosis complications was 7.2%, accounting for 1.75% events patient-years; when considering death from non-CV causes as competing events, the adjusted rate of major thrombosis was 2.2% patient-years, overall comparable with that of essential thrombocythemia (ET). Independent risk factors for fatal and nonfatal thrombosis were age, JAK2 mutational status and baseline leucocytosis.

Recently, somatic mutations in the gene encoding calreticulin (CALR) have been found in 50–70% of JAK2 and MPL-unmutated PMF and ET patients. In ET, CALR mutation confers a lower risk of thrombosis compared with JAK2 and MPL mutation.2, 3 Interestingly, CALR-mutational status in ET did not significantly impact the IPSET-thrombosis prognostic score.4 However, no information on the correlates of CALR mutation with the thrombotic risk in PMF is so far available.

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References

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Acknowledgements

TB, AR and AMV were supported by a grant from Associazione Italiana per la Ricerca sul Cancro (AIRC, Milano) ‘Special Program Molecular Clinical Oncology 5 × 1000’ to AGIMM (AIRC-Gruppo Italiano Malattie Mieloproliferative).

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Authors and Affiliations

  1. Hematology division, A.O. Papa Giovanni XXIII, Bergamo, Italy

    M C Finazzi, A Rambaldi, G Finazzi & T Barbui

  2. Research Foundation, A.O. Papa Giovanni XXIII, Bergamo, Italy

    A Carobbio & T Barbui

  3. Hematology Department, Hospital Clínic, IDIBAPS, University of Barcelona, Barcelona, Spain

    F Cervantes & I M Isola

  4. Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy

    A M Vannucchi & P Guglielmelli

  5. Center for the Study of Myelofibrosis, IRCCS Policlinico S. Matteo Foundation, Pavia, Italy

    G Barosi

Authors
  1. M C Finazzi
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  2. A Carobbio
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  3. F Cervantes
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  4. I M Isola
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  5. A M Vannucchi
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  6. P Guglielmelli
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  7. A Rambaldi
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  8. G Finazzi
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  9. G Barosi
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  10. T Barbui
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Correspondence to T Barbui.

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Finazzi, M., Carobbio, A., Cervantes, F. et al. CALR mutation, MPL mutation and triple negativity identify patients with the lowest vascular risk in primary myelofibrosis. Leukemia 29, 1209–1210 (2015). https://doi.org/10.1038/leu.2014.343

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