Hatalova, Antónia (26026342800)Antónia (26026342800)HatalovaSchwarz, Jiri (57200197190)Jiri (57200197190)SchwarzGotic, Mirjana (7004685432)Mirjana (7004685432)GoticPenka, Miroslav (7004517070)Miroslav (7004517070)PenkaHrubisko, Mikulas (7005725123)Mikulas (7005725123)HrubiskoKusec, Rajko (6603895241)Rajko (6603895241)KusecEgyed, Miklós (7003745502)Miklós (7003745502)EgyedGriesshammer, Martin (7004451358)Martin (7004451358)GriesshammerPodolak-Dawidziak, Maria (7004116603)Maria (7004116603)Podolak-DawidziakHellmann, Andrzej (7005850740)Andrzej (7005850740)HellmannKlymenko, Sergiy (8719752000)Sergiy (8719752000)KlymenkoNiculescu-Mizil, Emilia (6602358732)Emilia (6602358732)Niculescu-MizilPetrides, Petro E. (7005723256)Petro E. (7005723256)PetridesGrosicki, Sebastian (6506087019)Sebastian (6506087019)GrosickiSever, Matjaz (26024438200)Matjaz (26024438200)SeverCantoni, Nathan (27967498800)Nathan (27967498800)CantoniThiele, Jürgen (7202528008)Jürgen (7202528008)ThieleWolf, Dominik (9638732200)Dominik (9638732200)WolfGisslinger, Heinz (7005768562)Heinz (7005768562)Gisslinger2025-06-122025-06-122018https://doi.org/10.1111/ejh.13156https://www.scopus.com/inward/record.uri?eid=2-s2.0-85053386651&doi=10.1111%2fejh.13156&partnerID=40&md5=087bb895ee156bc8b21b431219cc08c7https://remedy.med.bg.ac.rs/handle/123456789/6143Objectives: 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-α 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. Conclusions: Greater understanding of PV is serving as a platform for new therapy development and treatment response predictors. © 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltdcytoreductive therapydiagnosismanagementmyeloproliferative neoplasmspolycythaemia verarecommendations