How we treat polycythemia vera

Abstract

Polycythemia vera is a disease known since ancient times, however, until recent decades, diagnosis was carried out by exclusion, and therapy was symptomatic. The discovery of the pathogenetic role of mutations in the Janus kinase II gene has led to the possibility of establishing a diagnosis based not only on morphology, but also on genetic verification and to the development of directed targeted therapy, which is much more effective than previously used methods. The introduction of molecular genetic screening led to the need for a differential diagnosis with familial erythrocytosis, and the lessons of the coronavirus pandemic revealed the presence in the population of a significant proportion of patients with erythrocytosis due to the carriage of gene polymorphisms associated with familial hemochromatosis. The article presents our own personalized algorithms for the diagnosis and treatment of polycythemia vera and the results of their use, demonstrating the possibility of a two-fold reduction in the incidence of thrombosis and an increase in overall survival.