Onkológia S2/2020

Polycytemia vera – how to treat further the patient after exhaustion of conventional therapy (hydroxyurea and interferon)

Purpose: Polycythemia vera (PV) is a myeloproliferative disease characterized by clonal stem cell proliferation, with most patients having a Janus kinase 2 (JAK2) mutation. It is characterized by erythrocytosis, an increase in the number of white blood cells and platelets. The disease is associated with a high risk of vascular complications and shortened life expectancy. Case: A 52-year-old patient with polyglobulia and recurrent transient ischemic attacks (TIA) was sent to our workplace where a diagnosis of polycythemia vera – high risk was made on the basis of examinations. We had to stop the initial treatment of IFN-α after 18 months for depression with suicidal tendencies. As the patient did not consent to cytoreductive therapy, antithrombotic drugs were prescribed to him. Due to recurrence of TIA, the patient agreed to hydroxyurea therapy. However, he did not tolerate this treatment either, so after a transitional period with phlebotomies we started the therapy with ruxolitinib, which brought the expected effect. The patient tolerates it well, continues treatment until now, without the need for dose reduction. Conclusion: PV is a chronic disease that shortens life not only by an incresed incidence of thromboembolic events, but also by development to postPV myelofibrosis and transition to AML. However, with proper treatment patients with PV can achieve near-normal life expectancy.

Keywords: polycytemia vera, interferon, hydroxyurea, phlebotomy, ruxolitinib