2018
Recommendations for the diagnosis and treatment of patients with polycythaemia vera
HATALOVA, Antónia, Jiri SCHWARZ, Mirjana GOTIC, Miroslav PENKA, Mikulas HRUBISKO et. al.Základní údaje
Originální název
Recommendations for the diagnosis and treatment of patients with polycythaemia vera
Autoři
HATALOVA, Antónia (703 Slovensko, garant), Jiri SCHWARZ (203 Česká republika), Mirjana GOTIC (688 Srbsko), Miroslav PENKA (203 Česká republika, domácí), Mikulas HRUBISKO (703 Slovensko), Rajko KUSEC (191 Chorvatsko), Miklos EGYED (348 Maďarsko), Martin GRIESSHAMMER (276 Německo), Maria PODOLAK-DAWIDZIAK (616 Polsko), Andrzej HELLMANN (616 Polsko), Sergiy KLYMENKO (804 Ukrajina), Emilia NICULESCU-MIZII (642 Rumunsko), Petro E. PETRIDES (276 Německo), Sebastian GROSICKI (616 Polsko), Matjaz SEVER (705 Slovinsko), Nathan CANTONI (756 Švýcarsko), Juergen THIELE (276 Německo), Dominik WOLF (40 Rakousko) a Heinz GISSLINGER (40 Rakousko)
Vydání
European Journal of Haematology, Hoboken, Wiley-Blackwell, 2018, 0902-4441
Další údaje
Jazyk
angličtina
Typ výsledku
Článek v odborném periodiku
Obor
30205 Hematology
Stát vydavatele
Spojené státy
Utajení
není předmětem státního či obchodního tajemství
Impakt faktor
Impact factor: 2.217
Kód RIV
RIV/00216224:14110/18:00104228
Organizační jednotka
Lékařská fakulta
UT WoS
000446560100011
Klíčová slova anglicky
cytoreductive therapy; diagnosis; management; myeloproliferative neoplasms; polycythaemia vera; recommendations
Příznaky
Mezinárodní význam, Recenzováno
Změněno: 10. 2. 2019 18:36, Soňa Böhmová
Anotace
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.