Onkológia 4/2019
Polycythemia vera – treatment options and today‘s practice
Polycythemia vera (PV) is Philadelphia chromosome-negative chronic myeloproliferative neoplasma (Ph- MPN), which hallmark is the clonal proliferation of hemopoetic stem cell in bone marrow with expanded red cell mass and dominant erythrocytosis in peripheral blood and/or spleen. PV is characterized by thromboembolic events, progression to myelofibrosis (MF), and transformation to acute leukemia (AL) with impaired survival due to all these complications. The primary goals of treatment for MPNs are to reduce the risk of thrombosis, alleviate systemic symptom burden (fatigue, pruritus, microvascular symptoms, splenomegaly) and prevent transformation to MF/AL. Currently available drugs for PV have not been shown to prolong survival or alter the natural history of the disease. At present, we continue with conservative management in low-risk PV (phlebotomy, aspirin) and with cytoreductive therapy in high-risk patients with first, second and third line drugs (hydroxyurea, interferon-alfa, pegylated interferon-alfa and JAK2 inhibitor therapy).
Keywords: polycythemia vera, thromboembolic events, progression, transformation, hydroxyurea, pegylated interferon-alfa, JAK1/2 inhibitor
