Prognostic impact of bone marrow fibrosis in primary myelofibrosis. A study of the AGIMM group on 490 patients

  • Paola Guglielmelli
    Department of Experimental and Clinical Medicine and DENOTHE Excellence Center CRIMM‐Center for Research and Innovation of Myeloproliferative Neoplasms, AOU Careggi, University of Florence Florence Italy
  • Giada Rotunno
    Department of Experimental and Clinical Medicine and DENOTHE Excellence Center CRIMM‐Center for Research and Innovation of Myeloproliferative Neoplasms, AOU Careggi, University of Florence Florence Italy
  • Annalisa Pacilli
    Department of Experimental and Clinical Medicine and DENOTHE Excellence Center CRIMM‐Center for Research and Innovation of Myeloproliferative Neoplasms, AOU Careggi, University of Florence Florence Italy
  • Elisa Rumi
    Department of Hematology Oncology Fondazione IRCCS Policlinico San Matteo Pavia Italy
  • Vittorio Rosti
    Center for the Study of Myelofibrosis, Research Lab of Biotechnology, IRCCS Policlinico San Matteo Foundation Pavia Italy
  • Federica Delaini
    Hematology and BMT Unit ASST Papa Giovanni XXIII Bergamo Italy
  • Margherita Maffioli
    Ospedale Di Circolo Fondazione Macchi Università Degli Studi Dell'Insubria Varese Viale Borri 57 Varese Italia
  • Tiziana Fanelli
    Department of Experimental and Clinical Medicine and DENOTHE Excellence Center CRIMM‐Center for Research and Innovation of Myeloproliferative Neoplasms, AOU Careggi, University of Florence Florence Italy
  • Alessandro Pancrazzi
    Department of Experimental and Clinical Medicine and DENOTHE Excellence Center CRIMM‐Center for Research and Innovation of Myeloproliferative Neoplasms, AOU Careggi, University of Florence Florence Italy
  • Lisa Pieri
    Department of Experimental and Clinical Medicine and DENOTHE Excellence Center CRIMM‐Center for Research and Innovation of Myeloproliferative Neoplasms, AOU Careggi, University of Florence Florence Italy
  • Rajmonda Fjerza
    Department of Experimental and Clinical Medicine and DENOTHE Excellence Center CRIMM‐Center for Research and Innovation of Myeloproliferative Neoplasms, AOU Careggi, University of Florence Florence Italy
  • Daniela Pietra
    Department of Hematology Oncology Fondazione IRCCS Policlinico San Matteo Pavia Italy
  • Daniela Cilloni
    Department of Clinical and Biological Sciences University of Turin San Luigi Hospital Turin Italy
  • Emanuela Sant'Antonio
    Department of Experimental and Clinical Medicine and DENOTHE Excellence Center CRIMM‐Center for Research and Innovation of Myeloproliferative Neoplasms, AOU Careggi, University of Florence Florence Italy
  • Silvia Salmoiraghi
    Hematology and BMT Unit ASST Papa Giovanni XXIII Bergamo Italy
  • Francesco Passamonti
    Ospedale Di Circolo Fondazione Macchi Università Degli Studi Dell'Insubria Varese Viale Borri 57 Varese Italia
  • Alessandro Rambaldi
    Hematology and BMT Unit ASST Papa Giovanni XXIII Bergamo Italy
  • Giovanni Barosi
    Center for the Study of Myelofibrosis, Research Lab of Biotechnology, IRCCS Policlinico San Matteo Foundation Pavia Italy
  • Tiziano Barbui
    Research Foundation Ospedale Papa Giovanni XXIII Bergamo Italy
  • Mario Cazzola
    Department of Hematology Oncology Fondazione IRCCS Policlinico San Matteo Pavia Italy
  • Alessandro M. Vannucchi
    Department of Experimental and Clinical Medicine and DENOTHE Excellence Center CRIMM‐Center for Research and Innovation of Myeloproliferative Neoplasms, AOU Careggi, University of Florence Florence Italy

抄録

<jats:p>The prognostic significance of bone marrow (BM) fibrosis grade in patients with primary myelofibrosis (PMF) is still debated. A fibrosis grade greater than 1 was shown to associate with higher risk of death, and addition of fibrosis grade to IPSS score resulted in a more accurate prediction of survival. The aim of this study was to analyze the prognostic impact of BM fibrosis in 490 patients with PMF, evaluated at diagnosis, molecularly annotated and with extensive follow‐up information. We found that fibrosis grade 2 and greater on a 0–3 scale was associated with clinical characteristics indicative of a more advanced disease, such as anemia, leukopenia, thrombocytopenia, constitutional symptoms, larger splenomegaly and a higher IPSS risk category. Patients with higher grade of fibrosis were also more likely to have additional somatic mutations in <jats:italic>ASXL1</jats:italic> and <jats:italic>EZH2</jats:italic>, that are prognostically adverse. Median survival was significantly reduced in patients with grade 2 and 3 fibrosis as compared with grade 1; this effect was maintained when analysis was restricted to younger patients. In multivariate analysis, fibrosis grade independently predicted for survival regardless of IPSS variables and mutational status; the adverse impact of fibrosis was noticeable especially in lower IPSS risk categories. Overall, results indicate that higher grades of fibrosis correlate with unique clinical and molecular aspects and represent an independent adverse variable in patients with PMF; these observations deserve confirmation in prospectively designed series of patients. Am. J. Hematol. 91:918–922, 2016. © 2016 Wiley Periodicals, Inc.</jats:p>

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