Recommendations for the diagnosis and treatment of patients with polycythaemia vera.
Eur J Haematol
; 2018 Jul 30.
Article
en En
| MEDLINE
| ID: mdl-30058088
ABSTRACT
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-α 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.
Texto completo:
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Bases de datos:
MEDLINE
Tipo de estudio:
Diagnostic_studies
/
Guideline
/
Prognostic_studies
Idioma:
En
Revista:
Eur J Haematol
Asunto de la revista:
HEMATOLOGIA
Año:
2018
Tipo del documento:
Article
País de afiliación:
Eslovaquia