Austrian recommendations for the management of essential thrombocythemia
- PDF / 516,532 Bytes
- 10 Pages / 595 x 842 pts (A4) Page_size
- 61 Downloads / 177 Views
Wien Klin Wochenschr https://doi.org/10.1007/s00508-020-01761-3
Austrian recommendations for the management of essential thrombocythemia 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
Received: 28 March 2020 / Accepted: 19 October 2020 © Springer-Verlag GmbH Austria, part of Springer Nature 2020
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.
V. Buxhofer-Ausch () Department of Internal Medicine I for Hematology with Stem Cell Transplantation, Hemostaseology and Medical Oncology, Ordensklinikum Linz Elisabethinen, Fadingerstraße 1, 4020 Linz, Austria [email protected]
M. T. Krauth · H. Gisslinger Department of Internal Medicine I, Division of Hematology and Blood Coagulation, Medical University of Vienna, Vienna, Austria
V. Buxhofer-Ausch · A. Petzer Medizinische Fakultät, Johannes Kepler Universität Linz, Altenberger Straße 69, 4040 Linz, Austria S. Heibl Department of Internal Medicine IV, Klinikum Wels-Grieskirchen, Wels, Austria T. Sliwa 3rd Medical Department, Hanusch Hospital, Vienna, Austria C. Beham-Schmid Institute of Pathology, Medical University Graz, Graz, Austria D. Wolf Division of Hematology and Oncology, Innsbruck Medical University, Innsbruck, Austria K. Geissler 5th Medical Department with Hematology, Oncology and Palliative Medicine, Hospital Hietzing, Vienna, Austria
K
Keywords Myeloproliferative neoplasms · Essential thrombocythemia · Management recommendations · Risk stratification · Treatment
P. Krippl Department of Internal Medicine with Hematology and Oncology, Steiermärkische Krankenanstaltengesellschaft m. b. H. Krankenhausverbund Feldbach-Fürstenfeld, Fürstenfeld, Austria A. Petzer Departments of Internal Medicine I for Hematology with Stem Cell Transplantation, Hemostaseology and Medical Oncology, Ordensklinikum Linz, Linz, Austria A. Wölfler Division of Hematology, Medical University of Graz, Graz, Austria T. Melchardt 3rd Medic
Data Loading...
