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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

We tested the hypothesis that high levels of CRP could predict the progression to acute leukemia and mortality in patients with primary or post-ET/PV MF. No financial interests in connection with this work have to be declared. After IRB approval, we prospectively reviewed the medical records of 184 patients with PMF (WHO 2008 diagnosis criteria)10 or post-ET/PV MF11 in three Italian centers, to assess the LT rate and survival. Blood was collected during a follow-up visit or retrieved from plasma archives. A total of 78 patients (42%) received cytoreductive therapy (hydroxyurea). High-sensitivity C-reactive protein (hs-CRP) was measured as previously described.9

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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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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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