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Characteristics of JAK2 unmutated erythrocytosis: Distinctive traits between polycythemia vera and non-polycythemia vera patients

  • Vincent Ernest
    Affiliations
    Laboratory of Hemostasis, Assistance Publique-Hôpitaux de Marseille (AP-HM), CHU La Timone, Marseille, France
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  • Norman Abbou
    Affiliations
    Biochemistry and Molecular Biology Laboratory, Assistance Publique-Hôpitaux de Marseille (AP-HM), CHU NORD, Marseille, France
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  • Antoine Tichadou
    Affiliations
    Hematology and Cellular Therapy Department, Assistance Publique-Hôpitaux de Marseille (AP-HM), CHU La Conception, Marseille, France
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  • Robin Arcani
    Correspondence
    Corresponding author at: Internal Medicine and Therapeutics Department, CHU La Timone, Assistance Publique-Hôpitaux de Marseille (AP-HM), Marseille, France.
    Affiliations
    Internal Medicine and Therapeutics Department, CHU La Timone, Assistance Publique-Hôpitaux de Marseille (AP-HM), Marseille, France

    Center for Cardiovascular and Nutrition Research (C2VN), INRA 1260, INSERM UMR_S 1263, Aix-Marseille University, Marseille, France
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  • Geoffroy Venton
    Affiliations
    Hematology and Cellular Therapy Department, Assistance Publique-Hôpitaux de Marseille (AP-HM), CHU La Conception, Marseille, France
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Published:September 09, 2022DOI:https://doi.org/10.1016/j.ejim.2022.09.004
      Polycythemia is a frequent reason for consultation, and is related to various etiologies. Erythropoietin (EPO) plays a major role in the physiological stimulation of red blood cell (RBC) progenitors’ differentiation and proliferation. EPO production depends on Hypoxia-Inducible Factors (HIF), which are oxygen-sensitive [
      • Haase V.H.
      Regulation of erythropoiesis by hypoxia-inducible factors.
      ]. In hypoxia conditions, stabilized HIF-2A promotes expression of the EPO gene. EPO binds to the EPO-receptor (EPOR) at the surface of RBC progenitors, thus stimulating their expansion [
      • Tóthová Z.
      • Tomc J.
      • Debeljak N.
      • Solár P.
      STAT5 as a key protein of erythropoietin signalization.
      ].

      Keywords

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