Recommendations for the diagnosis and treatment of patients with polycythaemia vera

Authors	HATALOVA Antónia 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 Juergen WOLF Dominik GISSLINGER Heinz
Year of publication	2018
Type	Article in Periodical
Magazine / Source	European Journal of Haematology
MU Faculty or unit	Faculty of Medicine
Citation
Doi	http://dx.doi.org/10.1111/ejh.13156
Keywords	cytoreductive therapy; diagnosis; management; myeloproliferative neoplasms; polycythaemia vera; recommendations
Description	ObjectivesTo present the Central European Myeloproliferative Neoplasm Organisation (CEMPO) treatment recommendations for polycythaemia vera (PV). MethodsDuring meetings held from 2015 through 2017, CEMPO discussed PV and its treatment and recent data. ResultsPV 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. ConclusionsGreater understanding of PV is serving as a platform for new therapy development and treatment response predictors.