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ORIGINAL RESEARCH article

Front. Hematol.
Sec. Blood Cancer
Volume 3 - 2024 | doi: 10.3389/frhem.2024.1492680
This article is part of the Research Topic Myeloproliferative Neoplasms: Updates for Patients and Healthcare Providers View all 4 articles

Distinct Clinico-Genomic Factors Drive Outcomes in Patients with Myelofibrosis and Disease-Related Anemia Running Head: Prognostic Correlates in Anemic Myelofibrosis Article Category: Original Article

Provisionally accepted
Somedeb Ball Somedeb Ball 1*Najla H Al Ali Najla H Al Ali 2Akriti Gupta Jain Akriti Gupta Jain 3Luis E Aguirre Luis E Aguirre 4Seongseok Yun Seongseok Yun 2Onyee Chan Onyee Chan 2Zhuoer Xie Zhuoer Xie 2David Sallman David Sallman 2Jeffrey Lancet Jeffrey Lancet 2Eric Padron Eric Padron 2Rami S Komrokji Rami S Komrokji 2Andrew T Kuykendall Andrew T Kuykendall 2
  • 1 Vanderbilt Ingram Cancer Center, Nashville, United States
  • 2 Moffitt Cancer Center, Tampa, Florida, United States
  • 3 Cleveland Clinic, Cleveland, Ohio, United States
  • 4 Dana–Farber Cancer Institute, Boston, Massachusetts, United States

The final, formatted version of the article will be published soon.

    BACKGROUND: Disease related anemia in myelofibrosis (MF) is common and prognostically detrimental. Anemia in MF poses a therapeutic challenge as it contributes to poor quality of life and often interferes with JAK inhibitor therapy. Still, the causes for anemia in MF are varied raising the question as to whether all patients with MF-related anemia should be viewed through the same prognostic lens.METHODS: In this retrospective study, we analyzed clinical and genomic data of patients with MF-related anemia using an institutional MF database. Anemia was defined as the requirement of red blood cell transfusions or a hemoglobin level of <10 g/dL at presentation. Multivariable analysis performed using Cox regression formed the basis of a proposed prognostic scoring system for patients with anemic MF. RESULTS: Among 739 patients with MF, 365 (49.5%) were anemic at presentation. Anemic patients were older, had lower platelet count, lower serum albumin, and higher ferritin level than non-anemic patients. The presence of a JAK2 mutation was less common, whereas mutations in U2AF1 and EZH2 were enriched in the anemic cohort. Blast phase transformation was more common in anemic patients. After a median follow up of 34.5 months, median overall survival (OS) was significantly shorter in anemic vs. non-anemic MF (30.2 vs. 73.9 months; p<0.01).Leukocytosis, thrombocytopenia, low serum albumin, and the presence of a mutation involving SRSF2 or TP53 were independent predictors of inferior OS in anemic MF on multivariable analysis. A proposed prognostic model including these factors stratified anemic MF cohort into low, intermediate, and high-risk categories, with median OS of 69, 37.7, and 11.6 months, respectively (p <0.01).CONCLUSIONS: Our study highlights the heterogeneity of patients with MF and anemia and identifies key prognostic correlates in this subgroup. Our proposed model may help guide therapeutic decision-making in this high-risk cohort.

    Keywords: Cytopenic myelofibrosis, Prognostic model, overall survival, albumin, Thrombocytopenia, SRSF2, TP53 Cytopenic myelofibrosis, SFSF2

    Received: 07 Sep 2024; Accepted: 04 Nov 2024.

    Copyright: © 2024 Ball, Al Ali, Jain, Aguirre, Yun, Chan, Xie, Sallman, Lancet, Padron, Komrokji and Kuykendall. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Somedeb Ball, Vanderbilt Ingram Cancer Center, Nashville, United States

    Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.