Universität zu Köln

Hatalova, Antonia, Schwarz, Jiri, Gotic, Mirjana, Penka, Miroslav, Hrubisko, Mikulas, Kusec, Rajko, Egyed, Miklos, Griesshammer, Martin, Podolak-Dawidziak, Maria, Hellmann, Andrzej, Klymenko, Sergiy ORCID: 0000-0002-9758-7316, Niculescu-Mizii, Emilia, Petrides, Petro E., Grosicki, Sebastian ORCID: 0000-0003-2644-1050, Sever, Matjaz, Cantoni, Nathan, Thiele, Juergen, Wolf, Dominik and Gisslinger, Heinz (2018). Recommendations for the diagnosis and treatment of patients with polycythaemia vera. Eur. J. Haematol., 101 (5). S. 654 - 665. HOBOKEN: WILEY. ISSN 1600-0609

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Abstract

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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Hatalova, Antonia UNSPECIFIED UNSPECIFIED UNSPECIFIED Schwarz, Jiri UNSPECIFIED UNSPECIFIED UNSPECIFIED Gotic, Mirjana UNSPECIFIED UNSPECIFIED UNSPECIFIED Penka, Miroslav UNSPECIFIED UNSPECIFIED UNSPECIFIED Hrubisko, Mikulas UNSPECIFIED UNSPECIFIED UNSPECIFIED Kusec, Rajko UNSPECIFIED UNSPECIFIED UNSPECIFIED Egyed, Miklos UNSPECIFIED UNSPECIFIED UNSPECIFIED Griesshammer, Martin UNSPECIFIED UNSPECIFIED UNSPECIFIED Podolak-Dawidziak, Maria UNSPECIFIED UNSPECIFIED UNSPECIFIED Hellmann, Andrzej UNSPECIFIED UNSPECIFIED UNSPECIFIED Klymenko, Sergiy UNSPECIFIED orcid.org/0000-0002-9758-7316 UNSPECIFIED Niculescu-Mizii, Emilia UNSPECIFIED UNSPECIFIED UNSPECIFIED Petrides, Petro E. UNSPECIFIED UNSPECIFIED UNSPECIFIED Grosicki, Sebastian UNSPECIFIED orcid.org/0000-0003-2644-1050 UNSPECIFIED Sever, Matjaz UNSPECIFIED UNSPECIFIED UNSPECIFIED Cantoni, Nathan UNSPECIFIED UNSPECIFIED UNSPECIFIED Thiele, Juergen UNSPECIFIED UNSPECIFIED UNSPECIFIED Wolf, Dominik UNSPECIFIED UNSPECIFIED UNSPECIFIED Gisslinger, Heinz UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-168609
DOI:	10.1111/ejh.13156
Journal or Publication Title:	Eur. J. Haematol.
Volume:	101
Number:	5
Page Range:	S. 654 - 665
Date:	2018
Publisher:	WILEY
Place of Publication:	HOBOKEN
ISSN:	1600-0609
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language WORLD-HEALTH-ORGANIZATION; QUALITY-OF-LIFE; ESSENTIAL THROMBOCYTHEMIA; RISK-FACTORS; MYELOPROLIFERATIVE DISORDERS; PRIMARY MYELOFIBROSIS; MOLECULAR RESPONSES; MYELOID NEOPLASMS; ACUTE-LEUKEMIA; ALLELE BURDEN Multiple languages Hematology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/16860