Onkológia S1/2022

Recommendations for the treatment of patients with polycythaemia vera in the Slovak Republic

Polycythemia vera (PV) is a chronic myeloproliferative neoplasm (MPN), which is associated with a shortened survival as a consequence of potentially fatal thrombotic complications, progression into myelofibrosis (MF) or an acute myeloid leukemia (AML). Thrombosis is historically the most common cause of death with PV and for this reason are the currently valid recommendations and prognosis models aimed at preventing trombotic complications. Nevertheless, the overall survival of the patients with PV remains shortened in comparison to the general populace. The course of PV may not be regarded as indolent. The progression of PV into MF, i.e. AML is a common cause of later morbidity and mortality, hence it is necessary to focus the treatment management of the patient on choosing and the sufficiently early timing of such treatment measures, that have potential to favourably affect the course of the disease. Currently valid recommendations do not always fulfill the clinical needs of the young, i.e. „low-risk patients“, for whom exists a lifelong danger of PV progressing into advanced stages. In spite of this, they are not being treated. Based on our knowledge from consultation activities in centres, we are aware of the inconsistencies in treatment practices outside haematology centres. This inconsistency in the management led us to develop Slovak recommendations that aim to increase availability of the treatment with the potential to prevent disease progression and the improvement of survival for every PV patient in Slovakia.

Keywords: myeloproliferative neoplasms, polycythaemia vera, treatment recommendations, risk stratification