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Elevated C-reactive protein is associated with shortened leukemia-free survival in patients with myelofibrosis

Leukemia volume 27, pages 2084–2086 (2013)Cite this article

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Myelofibrosis (MF) is a stem cell-derived neoplasm encompassing one of the chronic myeloproliferative neoplasms (MPN), and may present as primary (PMF) or secondary to polycythemia vera and essential thrombocythemia (post-PV/ET MF). The cardinal pathophysiological feature of these MPN is dysregulated JAK2 signaling, most commonly due to the activating V617F mutation in JAK2 itself or mediated by other complex genetic and epigenetic mechanisms.1, 2 The course of MF is characterized by progressive anemia, splenomegaly and constitutional symptoms and by a median survival estimated at 4–6 years.3 Causes of death include leukemia transformation (LT), progressive disease with bone marrow failure and complications from thrombosis, bleeding, infections and portal hypertension.4 The risk factors for leukemic transformation of MF are not fully understood and have been debated in the literature.2, 5, 6 Recently, a possible role has been attributed to a chronic inflammation state characterized by increased plasma levels of several inflammatory biomarkers. IL-8 and IL-2R were associated independently with phenotypic manifestations and more importantly with inferior overall and leukemia-free survival.7 Moreover, increased levels of short pentraxin C-reactive protein (CRP), a marker of systemic inflammation produced in hepatocytes following stimulation by cytokines, especially IL-6, have been found in MF and in patients with ET and PV.8, 9

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References

  1. Vainchenker W, Favale F . Myelofibrosis, JAK2 inhibitors and erythropoiesis. Leukemia 2013; 27: 1219–1223.

    Article  CAS  PubMed  Google Scholar 

  2. Vannucchi AM, Lasho TL, Guglielmelli P, Biamonte F, Pardanani A, Pereira A et al. Mutations and prognosis in primary myelofibrosis. Leukemia 2013; 27: 1861–1869.

    Article  CAS  PubMed  Google Scholar 

  3. Cervantes F, Dupriez B, Passamonti F, Vannucchi AM, Morra E, Reilly JT et al. Improving survival trends in primary myelofibrosis: an international study. J Clin Oncol 2012; 30: 2981–2987.

    Article  PubMed  Google Scholar 

  4. Tefferi A . Primary myelofibrosis: 2013 update on diagnosis, risk-stratification, and management. Am J Hematol. 2013; 88: 141–150.

    Article  CAS  PubMed  Google Scholar 

  5. Tefferi A, Lasho TL, Huang J, Finke C, Mesa RA, Li CY et al. Low JAK2V617F allele burden in primary myelofibrosis, compared to either a higher allele burden or unmutated status, is associated with inferior overall and leukemia-free survival. Leukemia 2008; 22: 756–761.

    Article  CAS  PubMed  Google Scholar 

  6. Poletto V, Rosti V, Villani L, Catarsi P, Carolei A, Campanelli R et al. A3669G polymorphism of glucocorticoid receptor is a susceptibility allele for primary myelofibrosis and contributes to phenotypic diversity and blast transformation. Blood 2012; 120: 3112–3117.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Tefferi A, Vaidya R, Caramazza D, Finke C, Lasho T, Pardanani A . Circulating interleukin (IL)-8, IL-2R, IL-12, and IL-15 levels are independently prognostic in primary myelofibrosis: a comprehensive cytokine profiling study. J Clin Oncol 2011; 29: 1356–1363.

    Article  CAS  PubMed  Google Scholar 

  8. Verstovsek S, Mesa RA, Gotlib J, Levy RS, Gupta V, DiPersio JF et al. A double-blind, placebo-controlled trial of ruxolitinib for myelofibrosis. N Engl J Med 2012; 366: 799–807.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Barbui T, Carobbio A, Finazzi G, Vannucchi AM, Barosi G, Antonioli E et al. Inflammation and thrombosis in essential thrombocythemia and polycythemia vera: different role of C-reactive protein and pentraxin 3. Haematologica 2011; 96: 315–318.

    Article  CAS  PubMed  Google Scholar 

  10. Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood 2009; 114: 937–951.

    Article  CAS  PubMed  Google Scholar 

  11. Barosi G, Mesa RA, Thiele J, Cervantes F, Campbell PJ, Verstovsek S et al. Proposed criteria for the diagnosis of post-polycythemia vera and post-essential thrombocythemia myelofibrosis: a consensus statement from the international working group for myelofibrosis research and treatment. Leukemia 2008; 22: 437–438.

    Article  CAS  PubMed  Google Scholar 

  12. Harrell FE Jr, Lee KL, Mark DB . Multivariable prognostic models: issues in developing models, evaluating assumptions and adequacy, and measuring and reducing errors. Stat Med 1996; 15: 361–387.

    Article  PubMed  Google Scholar 

  13. Tefferi A, Pardanani A, Gangat N, Begna KH, Hanson CA, Van Dyke DL et al. Leukemia risk models in primary myelofibrosis: an International Working Group study. Leukemia 2012; 26: 1439–1441.

    Article  CAS  PubMed  Google Scholar 

  14. Hasselbalch HC . Chronic inflammation as a promotor of mutagenesis in essential thrombocythemia, polycythemia vera and myelofibrosis. A human inflammation model for cancer development? Leuk Res 2013; 37: 214–220.

    Article  CAS  PubMed  Google Scholar 

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Acknowledgements

This study was supported by a special grant from Associazione Italiana per la Ricerca sul Cancro—‘AIRC 5 per Mille’—to AGIMM, ‘AIRC-Gruppo Italiano Malattie Mieloproliferative’ (#1005); for a description of the AGIMM project, see www.progettoagimm.it).

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Authors and Affiliations

  1. Fondazione per la Ricerca Ospedale Maggiore (FROM), Ospedale Papa Giovanni XXIII, Bergamo, Italy

    T Barbui & A Carobbio

  2. Divisione di Ematologia, Ospedale Papa Giovanni XXIII, Bergamo, Italy

    T Barbui, G Finazzi, S Salmoiraghi & A Rambaldi

  3. Dipartimento di Medicina Sperimentale e Clinica, Università degli studi di Firenze, Florence, Italy

    P Guglielmelli & A M Vannucchi

  4. Laboratorio di Epidemiologia Clinica e Centro per lo Studio della Mielofibrosi, Fondazione IRCCS Policlinico S Matteo, Pavia, Italy

    V Rosti & G Barosi

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  1. T Barbui
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  2. A Carobbio
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  3. G Finazzi
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  4. P Guglielmelli
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  7. A Rambaldi
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  8. A M Vannucchi
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  9. G Barosi
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Correspondence to T Barbui.

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Barbui, T., Carobbio, A., Finazzi, G. et al. Elevated C-reactive protein is associated with shortened leukemia-free survival in patients with myelofibrosis. Leukemia 27, 2084–2086 (2013). https://doi.org/10.1038/leu.2013.207

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