Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Buxhofer-Ausch, V; Heibl, S; Sliwa, T; Beham-Schmid, C; Wolf, D; Geissler, K; Krauth, MT; Krippl, P; Petzer, A; Wölfler, A; Melchardt, T; Gisslinger, H.
Austrian recommendations for the management of essential thrombocythemia.
Wien Klin Wochenschr. 2021; 133(1-2):52-61
Doi: 10.1007/s00508-020-01761-3
Web of Science
PubMed
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- Co-Autor*innen der Med Uni Graz
- Beham-Schmid Christine
- Krippl Peter
- Wölfler Albert
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- 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.
- Find related publications in this database (using NLM MeSH Indexing)
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- Humans -
- Mutation -
- Myeloproliferative Disorders -
- Polycythemia Vera -
- Primary Myelofibrosis -
- Thrombocythemia, Essential - diagnosis
- Thrombocythemia, Essential - drug therapy
- Thrombocythemia, Essential - genetics
- Find related publications in this database (Keywords)
- Myeloproliferative neoplasms
- Essential thrombocythemia
- Management recommendations
- Risk stratification
- Treatment