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.

Základní údaje

Originální název

Recommendations for the diagnosis and treatment of patients with polycythaemia vera

Autoři

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 a Heinz GISSLINGER

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

Označené pro přenos do RIV

Ano

Kód RIV

RIV/00216224:14110/18:00104228

Organizační jednotka

Lékařská fakulta

EID Scopus

Klíčová slova anglicky

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

Štítky

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.