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. 2021 Jan;133(1-2):52-61.
doi: 10.1007/s00508-020-01761-3. Epub 2020 Nov 19.

Austrian recommendations for the management of essential thrombocythemia

Veronika Buxhofer-Ausch  1   2 , Sonja Heibl  3 , Thamer Sliwa  4 , Christine Beham-Schmid  5 , Dominik Wolf  6 , Klaus Geissler  7 , Maria Theresa Krauth  8 , Peter Krippl  9 , Andreas Petzer  10   11 , Albert Wölfler  12 , Thomas Melchardt  13 , Heinz Gisslinger  8
Affiliations

Austrian recommendations for the management of essential thrombocythemia

Veronika Buxhofer-Ausch et al. Wien Klin Wochenschr. 2021 Jan.

Abstract

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.

Keywords: Essential thrombocythemia; Management recommendations; Myeloproliferative neoplasms; Risk stratification; Treatment.

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Comment in

  • Acetylsalicylic acid in essential thrombocythemia.
    Scavone M, Podda GM, Bossi E, Paroni R, Cattaneo M. Scavone M, et al. Wien Klin Wochenschr. 2022 Jan;134(1-2):89-90. doi: 10.1007/s00508-021-01814-1. Epub 2021 Feb 3. Wien Klin Wochenschr. 2022. PMID: 33537841 No abstract available.

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