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Hatalova, Antonia; Schwarz, Jiri; Gotic, Mirjana; Penka, Miroslav; Hrubisko, Mikulas; Kusec, Rajko; Egyed, Miklos; Griesshammer, Martin; Podolak-Dawidziak, Maria; Hellmann, Andrzej; Klymenko, Sergiy; Niculescu-Mizii, Emilia; Petrides, Petro E.; Grosicki, Sebastian; Sever, Matjaz; Cantoni, Nathan; Thiele, Jürgen; Wolf, Dominik; Gisslinger, Heinz (2018): Recommendations for the diagnosis and treatment of patients with polycythaemia vera. In: European Journal of Haematology, Vol. 101, No. 5: pp. 654-664
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Abstract

Objective: To present the Central European Myeloproliferative Neoplasm Organisation (CEMPO) treatment recommendations for polycythaemia vera (PV). Methods: During meetings held from 2015 through 2017, CEMPO discussed PV and its treatment and recent data. Results: PV is associated with increased risks of thrombosis/thrombo-haemorrhagic complications, fibrotic progression and leukaemic transformation. Presence of Janus kinase (JAK)-2 gene mutations is a diagnostic marker and standard diagnostic criterion. World Health Organization 2016 diagnostic criteria for PV, focusing on haemoglobin levels and bone marrow morphology, are mandatory. PV therapy aims at managing long-term risks of vascular complications and progression towards transformation to acute myeloid leukaemia and myelodysplastic syndrome. Risk stratification for thrombotic complications guides therapeutic decisions. Low-risk patients are treated first line with low-dose aspirin and phlebotomy. Cytoreduction is considered for low-risk (phlebotomy intolerance, severe/progressive symptoms, cardiovascular risk factors) and high-risk patients. Hydroxyurea is suspected of leukaemogenic potential. IFN- has demonstrated efficacy in many clinical trials;its pegylated form is best tolerated, enabling less frequent administration than standard interferon. Ropeginterferon alfa-2b has been shown to be more efficacious than hydroxyurea. JAK1/JAK2 inhibitor ruxolitinib is approved for hydroxyurea resistant/intolerant patients. Conclusion: sGreater understanding of PV is serving as a platform for new therapy development and treatment response predictors.