Recommendations for the diagnosis and treatment of patients with polycythaemia vera
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11110%2F18%3A10388852" target="_blank" >RIV/00216208:11110/18:10388852 - isvavai.cz</a>
Alternative codes found
RIV/00216224:14110/18:00104228 RIV/65269705:_____/18:00070056 RIV/00023736:_____/18:00012424
Result on the web
<a href="https://doi.org/10.1111/ejh.13156" target="_blank" >https://doi.org/10.1111/ejh.13156</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1111/ejh.13156" target="_blank" >10.1111/ejh.13156</a>
Alternative languages
Result language
angličtina
Original language name
Recommendations for the diagnosis and treatment of patients with polycythaemia vera
Original language description
Objectives: 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. Conclusions: Greater understanding of PV is serving as a platform for new therapy development and treatment response predictors.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30205 - Hematology
Result continuities
Project
—
Continuities
V - Vyzkumna aktivita podporovana z jinych verejnych zdroju
Others
Publication year
2018
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
European Journal of Haematology
ISSN
0902-4441
e-ISSN
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Volume of the periodical
101
Issue of the periodical within the volume
5
Country of publishing house
GB - UNITED KINGDOM
Number of pages
11
Pages from-to
654-664
UT code for WoS article
000446560100011
EID of the result in the Scopus database
2-s2.0-85053386651