Austrian recommendations for the management of essential thrombocythemia
| Autor: | Thomas Melchardt, Thamer Sliwa, Veronika Buxhofer-Ausch, Heinz Gisslinger, Sonja Heibl, Andreas L. Petzer, Klaus Geissler, Peter Krippl, Christine Beham-Schmid, Dominik Wolf, Albert Wölfler, Maria Theresa Krauth |
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| Rok vydání: | 2020 |
| Předmět: |
Oncology
medicine.medical_specialty Disease 030204 cardiovascular system & hematology 03 medical and health sciences 0302 clinical medicine Polycythemia vera hemic and lymphatic diseases Internal medicine Humans Medicine 030212 general & internal medicine Myelofibrosis Polycythemia Vera Myeloproliferative Disorders Janus kinase 2 biology business.industry Essential thrombocythemia food and beverages General Medicine Anagrelide medicine.disease Thrombosis Primary Myelofibrosis Austria Mutation biology.protein business Calreticulin Thrombocythemia Essential medicine.drug |
| Zdroj: | Wiener klinische Wochenschrift. 133:52-61 |
| ISSN: | 1613-7671 0043-5325 |
| DOI: | 10.1007/s00508-020-01761-3 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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