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19.11.2020 | original article

Austrian recommendations for the management of essential thrombocythemia

verfasst von: Veronika Buxhofer-Ausch, Sonja Heibl, Thamer Sliwa, Christine Beham-Schmid, Dominik Wolf, Klaus Geissler, Maria Theresa Krauth, Peter Krippl, Andreas Petzer, Albert Wölfler, Thomas Melchardt, Heinz Gisslinger

Erschienen in: Wiener klinische Wochenschrift | Ausgabe 1-2/2021

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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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Metadaten
Titel
Austrian recommendations for the management of essential thrombocythemia
verfasst von
Veronika Buxhofer-Ausch
Sonja Heibl
Thamer Sliwa
Christine Beham-Schmid
Dominik Wolf
Klaus Geissler
Maria Theresa Krauth
Peter Krippl
Andreas Petzer
Albert Wölfler
Thomas Melchardt
Heinz Gisslinger
Publikationsdatum
19.11.2020
Verlag
Springer Vienna
Erschienen in
Wiener klinische Wochenschrift / Ausgabe 1-2/2021
Print ISSN: 0043-5325
Elektronische ISSN: 1613-7671
DOI
https://doi.org/10.1007/s00508-020-01761-3