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Austrian recommendations for the management of essential thrombocythemia

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Summary

According to the World Health Organization (WHO) classification, essential (primary) thrombocythemia (ET) is one of several Bcr-Abl negative chronic myeloproliferative neoplasms (MPN). The classical term MPN covers the subcategories of MPN: ET, polycythemia vera (PV), primary myelofibrosis (PMF), and prefibrotic PMF (pPMF). ET is marked by clonal proliferation of hematopoietic stem cells, leading to a chronic overproduction of platelets. At the molecular level a JAK2 (Janus Kinase 2), calreticulin, or MPL mutation is found in the majority of patients. Typical ongoing complications of the disease include thrombosis and hemorrhage. Primary and secondary prevention of these complications can be achieved with platelet function inhibitors and various cytoreductive drugs including anagrelide, hydroxyurea and interferon. After a long follow up, in a minority of ET patients the disease transforms into post-ET myelofibrosis or secondary leukemia. Overall, life expectancy with ET is only slightly decreased.

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Authors and Affiliations

  1. Department of Internal Medicine I for Hematology with Stem Cell Transplantation, Hemostaseology and Medical Oncology, Ordensklinikum Linz Elisabethinen, Fadingerstraße 1, 4020, Linz, Austria

    Veronika Buxhofer-Ausch

  2. Medizinische Fakultät, Johannes Kepler Universität Linz, Altenberger Straße 69, 4040, Linz, Austria

    Veronika Buxhofer-Ausch & Andreas Petzer

  3. Department of Internal Medicine IV, Klinikum Wels-Grieskirchen, Wels, Austria

    Sonja Heibl

  4. 3rd Medical Department, Hanusch Hospital, Vienna, Austria

    Thamer Sliwa

  5. Institute of Pathology, Medical University Graz, Graz, Austria

    Christine Beham-Schmid

  6. Division of Hematology and Oncology, Innsbruck Medical University, Innsbruck, Austria

    Dominik Wolf

  7. 5th Medical Department with Hematology, Oncology and Palliative Medicine, Hospital Hietzing, Vienna, Austria

    Klaus Geissler

  8. Department of Internal Medicine I, Division of Hematology and Blood Coagulation, Medical University of Vienna, Vienna, Austria

    Maria Theresa Krauth & Heinz Gisslinger

  9. Department of Internal Medicine with Hematology and Oncology, Steiermärkische Krankenanstaltengesellschaft m. b. H. Krankenhausverbund Feldbach-Fürstenfeld, Fürstenfeld, Austria

    Peter Krippl

  10. Departments of Internal Medicine I for Hematology with Stem Cell Transplantation, Hemostaseology and Medical Oncology, Ordensklinikum Linz, Linz, Austria

    Andreas Petzer

  11. Division of Hematology, Medical University of Graz, Graz, Austria

    Albert Wölfler

  12. 3rd Medical Department with Hematology and Medical Oncology, Hemostaseology, Rheumatology and Infectiology, Laboratory for Immunological and Molecular Cancer Research, Paracelsus Medical University Hospital Salzburg, Salzburg, Austria

    Thomas Melchardt

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Correspondence to Veronika Buxhofer-Ausch.

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V. Buxhofer-Ausch, S. Heibl, T. Sliwa, C. Beham-Schmid, D. Wolf, K. Geissler, M.T. Krauth, P. Krippl, A. Petzer, A. Wölfler, T. Melchardt, and H. Gisslinger declare that they have no competing interests.

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Buxhofer-Ausch, V., Heibl, S., Sliwa, T. et al. Austrian recommendations for the management of essential thrombocythemia. Wien Klin Wochenschr 133, 52–61 (2021). https://doi.org/10.1007/s00508-020-01761-3

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