Austrian recommendations for the management of essential thrombocythemia.

Wiener klinische Wochenschrift
Veronika Buxhofer-AuschHeinz Gisslinger

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.

References

Jul 8, 2005·The New England Journal of Medicine·Claire N HarrisonUNKNOWN United Kingdom Medical Research Council Primary Thrombocythemia 1 Study
Jul 1, 2006·Seminars in Thrombosis and Hemostasis·Martin GriesshammerClaire M Harrison
May 26, 2007·Seminars in Thrombosis and Hemostasis·Ayalew Tefferi, Michelle Elliott
Jul 12, 2008·Blood Reviews·Martin GriesshammerTiziano Barbui
Jul 13, 2011·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Tiziano BarbuiAyalew Tefferi
Sep 14, 2011·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Jean-Jacques KiladjianJean-Didier Rain
Feb 5, 2013·Expert Review of Hematology·Richard T SilverHans Carl Hasselbalch
Dec 12, 2013·The New England Journal of Medicine·Thorsten KlampflRobert Kralovics
Dec 25, 2013·Blood·Elisa RumiUNKNOWN Associazione Italiana per la Ricerca sul Cancro Gruppo Italiano Malattie Mieloproliferative Investigators
Dec 29, 2013·Blood·Giada RotunnoUNKNOWN Associazione Italiana per la Ricerca sul Cancro Gruppo Italiano Malattie Mieloproliferative Investigators
Jan 23, 2015·American Journal of Hematology·Ayalew Tefferi, Tiziano Barbui
Apr 9, 2015·Leukemia Research·Giovanni TortorellaUNKNOWN Gimema Foundation project “Registro Italiano Trombocitemie (RIT)”
Jul 3, 2015·Therapeutic Advances in Hematology·Gunnar Birgegård
Nov 22, 2016·Blood Advances·Ayalew TefferiAlessandro M Vannucchi
Apr 1, 2018·European Journal of Haematology·Veronika Buxhofer-AuschHeinz Gisslinger
Jul 18, 2018·Translational Research : the Journal of Laboratory and Clinical Medicine·Thomas GremmelSimon Panzer
Aug 8, 2018·Expert Review of Hematology·Martin GriesshammerKai Wille

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Citations

Feb 5, 2021·Wiener klinische Wochenschrift·Mariangela ScavoneMarco Cattaneo

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