Recommendations for the diagnosis and treatment of patients
with polycythaemia vera

J 2018

Recommendations for the diagnosis and treatment of patients with polycythaemia vera

HATALOVA, Antónia, Jiri SCHWARZ, Mirjana GOTIC, Miroslav PENKA, Mikulas HRUBISKO et. al.

Basic information

Original name

Recommendations for the diagnosis and treatment of patients with polycythaemia vera

Authors

HATALOVA, Antónia (703 Slovakia, guarantor), Jiri SCHWARZ (203 Czech Republic), Mirjana GOTIC (688 Serbia), Miroslav PENKA (203 Czech Republic, belonging to the institution), Mikulas HRUBISKO (703 Slovakia), Rajko KUSEC (191 Croatia), Miklos EGYED (348 Hungary), Martin GRIESSHAMMER (276 Germany), Maria PODOLAK-DAWIDZIAK (616 Poland), Andrzej HELLMANN (616 Poland), Sergiy KLYMENKO (804 Ukraine), Emilia NICULESCU-MIZII (642 Romania), Petro E. PETRIDES (276 Germany), Sebastian GROSICKI (616 Poland), Matjaz SEVER (705 Slovenia), Nathan CANTONI (756 Switzerland), Juergen THIELE (276 Germany), Dominik WOLF (40 Austria) and Heinz GISSLINGER (40 Austria)

Edition

European Journal of Haematology, Hoboken, Wiley-Blackwell, 2018, 0902-4441

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30205 Hematology

Country of publisher

United States of America

Confidentiality degree

není předmětem státního či obchodního tajemství

Impact factor

Impact factor: 2.217

RIV identification code

RIV/00216224:14110/18:00104228

Organization unit

Faculty of Medicine

DOI

http://dx.doi.org/10.1111/ejh.13156

UT WoS

000446560100011

Keywords in English

cytoreductive therapy; diagnosis; management; myeloproliferative neoplasms; polycythaemia vera; recommendations

Abstract

V originále

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.

Citovat

HATALOVA, Antónia, Jiri SCHWARZ, Mirjana GOTIC, Miroslav PENKA, Mikulas HRUBISKO, Rajko KUSEC, Miklos EGYED, Martin GRIESSHAMMER, Maria PODOLAK-DAWIDZIAK, Andrzej HELLMANN, Sergiy KLYMENKO, Emilia NICULESCU-MIZII, Petro E. PETRIDES, Sebastian GROSICKI, Matjaz SEVER, Nathan CANTONI, Juergen THIELE, Dominik WOLF and Heinz GISSLINGER. Recommendations for the diagnosis and treatment of patients with polycythaemia vera. European Journal of Haematology. Hoboken: Wiley-Blackwell, 2018, vol. 101, No 5, p. 654-664. ISSN 0902-4441. Available from: https://dx.doi.org/10.1111/ejh.13156.

@article{1456276,
   author = {Hatalova, Antónia and Schwarz, Jiri and Gotic, Mirjana and Penka, Miroslav and Hrubisko, Mikulas and Kusec, Rajko and Egyed, Miklos and Griesshammer, Martin and PodolakandDawidziak, Maria and Hellmann, Andrzej and Klymenko, Sergiy and NiculescuandMizii, Emilia and Petrides, Petro E. and Grosicki, Sebastian and Sever, Matjaz and Cantoni, Nathan and Thiele, Juergen and Wolf, Dominik and Gisslinger, Heinz},
   article_location = {Hoboken},
   article_number = {5},
   doi = {http://dx.doi.org/10.1111/ejh.13156},
   keywords = {cytoreductive therapy; diagnosis; management; myeloproliferative neoplasms; polycythaemia vera; recommendations},
   language = {eng},
   issn = {0902-4441},
   journal = {European Journal of Haematology},
   title = {Recommendations for the diagnosis and treatment of patients with polycythaemia vera},
   volume = {101},
   year = {2018}
}

TY  - JOUR
ID  - 1456276
AU  - 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
PY  - 2018
TI  - Recommendations for the diagnosis and treatment of patients with polycythaemia vera
JF  - European Journal of Haematology
VL  - 101
IS  - 5
SP  - 654-664
EP  - 654-664
PB  - Wiley-Blackwell
SN  - 09024441
KW  - cytoreductive therapy
KW  - diagnosis
KW  - management
KW  - myeloproliferative neoplasms
KW  - polycythaemia vera
KW  - recommendations
N2  - 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.
ER  -

HATALOVA, Antónia, Jiri SCHWARZ, Mirjana GOTIC, Miroslav PENKA, Mikulas HRUBISKO, Rajko KUSEC, Miklos EGYED, Martin GRIESSHAMMER, Maria PODOLAK-DAWIDZIAK, Andrzej HELLMANN, Sergiy KLYMENKO, Emilia NICULESCU-MIZII, Petro E. PETRIDES, Sebastian GROSICKI, Matjaz SEVER, Nathan CANTONI, Juergen THIELE, Dominik WOLF and Heinz GISSLINGER. Recommendations for the diagnosis and treatment of patients with polycythaemia vera. \textit{European Journal of Haematology}. Hoboken: Wiley-Blackwell, 2018, vol.~101, No~5, p.~654-664. ISSN~0902-4441. Available from: https://dx.doi.org/10.1111/ejh.13156.

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