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Long-standing thrombocytosis often precedes thromboembolic complications heralding the diagnosis of essential thrombocythemia

  • Author Footnotes
    1 These authors contributed equally to this work.
    Gregoire Stalder
    Footnotes
    1 These authors contributed equally to this work.
    Affiliations
    Service and Central Laboratory of Hematology, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL), Rue du Bugnon 46, Lausanne CH-1011, Switzerland

    Service of Hematology and Laboratory of Hematology, Institut Central des Hôpitaux, Hôpital du Valais, Av. du Grand-Champsec 86, Sion CH-1951, Switzerland
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  • Author Footnotes
    1 These authors contributed equally to this work.
    William Reis Da Silva
    Footnotes
    1 These authors contributed equally to this work.
    Affiliations
    Faculty of Biology and Medicine, University of Lausanne, rue du Bugnon 21, Lausanne CH-1011, Switzerland
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  • Amandine Segot
    Affiliations
    Service and Central Laboratory of Hematology, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL), Rue du Bugnon 46, Lausanne CH-1011, Switzerland
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  • Sabine Blum
    Affiliations
    Service and Central Laboratory of Hematology, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL), Rue du Bugnon 46, Lausanne CH-1011, Switzerland
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  • Francesco Grandoni
    Affiliations
    Service and Central Laboratory of Hematology, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL), Rue du Bugnon 46, Lausanne CH-1011, Switzerland
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  • Lorenzo Alberio
    Correspondence
    Corresponding author.
    Affiliations
    Service and Central Laboratory of Hematology, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL), Rue du Bugnon 46, Lausanne CH-1011, Switzerland
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  • Author Footnotes
    1 These authors contributed equally to this work.
Published:September 15, 2022DOI:https://doi.org/10.1016/j.ejim.2022.09.002
      Myeloproliferative neoplasms (MPN) are clonal diseases of hematopoietic stem cells characterized by the hyperplasia of one or more myeloid cell lines that retain their capacity for terminal differentiation [
      • Arber D.A.
      • Orazi A.
      • Hasserjian R.
      • Thiele J.
      • Borowitz M.J.
      • Le Beau M.M.
      • et al.
      The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia.
      ]. Three entities that share overlapping but distinct clinical and laboratory characteristics are referred to as Philadelphia chromosome-negative (or BCR-ABL-negative) MPN: polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). The incidence of ET in Europe is estimated to be 0.4 to 1.7 per 100,000 person-years [
      • Moulard O.
      • Mehta J.
      • Fryzek J.
      • Olivares R.
      • Iqbal U.
      • Mesa R.A.
      Epidemiology of myelofibrosis, essential thrombocythemia, and polycythemia vera in the European Union.
      ]. As all BCR-ABL-negative MPN, ET carries an increased risk of thrombosis and bleeding. A thromboembolic event can complicate the course of ET but more frequently can be its mode of presentation [
      • Barbui T.
      • Finazzi G.
      • Falanga A.
      Myeloproliferative neoplasms and thrombosis.
      ]. The risk of arterial and venous thrombotic events in patients with MPN is estimated to be respectively 4.9 and 6.7 times higher than in the general population [
      • Hultcrantz M.
      • Andersson T.M.L.
      • Landgren O.
      • Derolf A.R.
      • Dickman P.W.
      • Björkholm M.
      • et al.
      Risk of arterial and venous thrombosis in 11,155 patients with myeloproliferative neoplasms and 44,620 matched controls; a population-based study.
      ]. The overall risk of arterial or venous thrombosis in ET varies between 12% and 21% [
      • Radaelli F.
      • Colombi M.
      • Calori R.
      • Zilioli V.R.
      • Bramanti S.
      • Iurlo A.
      • et al.
      Analysis of risk factors predicting thrombotic and/or haemorrhagic complications in 306 patients with essential thrombocythemia.
      ,
      • Girodon F.
      • Bonicelli G.
      • Schaeffer C.
      • Mounier M.
      • Carillo S.
      • Lafon I.
      • et al.
      Significant increase in the apparent incidence of essential thrombocythemia related to new WHO diagnostic criteria: a population-based study.
      ,
      • Carobbio A.
      • Thiele J.
      • Passamonti F.
      • Rumi E.
      • Ruggeri M.
      • Rodeghiero F.
      • et al.
      Risk factors for arterial and venous thrombosis in WHO-defined essential thrombocythemia: an international study of 891 patients.
      ]. Of note, arterial and venous thrombosis are observed in 14% and 8% of patients before or at ET diagnosis and may occur in another 15% and 8% of patients during a median follow-up of 9.9 years [
      • Szuber N.
      • Mudireddy M.
      • Nicolosi M.
      • Penna D.
      • Vallapureddy R.R.
      • Lasho T.L.
      • et al.
      3023 mayo clinic patients with myeloproliferative neoplasms: risk-stratified comparison of survival and outcomes data among disease subgroups.
      ]. Our clinical experience as well as published data indicate that thromboembolic events are frequent at the moment of diagnosis, but may also occur long before the presence of ET is recognized [
      • Kaifie A.
      • Kirschner M.
      • Wolf D.
      • Maintz C.
      • Hänel M.
      • Gattermann N.
      • et al.
      Bleeding, thrombosis, and anticoagulation in myeloproliferative neoplasms (MPN): analysis from the German SAL-MPN-registry.
      ].

      Keywords

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