The original experience of observation and treatment of patients with polycythemia vera

Polycythemia Vera (PV) is a chronic myeloproliferative disorder characterized by predominant proliferation of myeloid germ of hemopoiesis, which is based on the defeat of the stem cell in the presence of somatic mutations in the gene of Janus kinas ( JAK2 ) receptors of cytokines. It is typical for PV possible development of extramedullary hematopoiesis, the risk of thrombotic complications, the outcome in myelofibrosis or transformation to acute leukemia. This article presents the results of our own experience of observation and treatment of 252 patients with PV, who was observed in consultative outpatient Department of the Russian research Institute of Hematology and transfusion medicine in the period from 1990 to 2014. Diagnosis of PV is based on the clinical analysis of blood, morphological and histological study of bone marrow, the detection of JAK2V617F mutation. The main clinical manifestations of PV were: plethora, headaches, dizziness, weakness, pruritus, joint pain, erythromelalgia. A significant deviation from the norm were revealed in the indicators of hemoglobin, erythrocytes, hematocrit, platelets, white blood cells. Almost all patients had JAK2 mutation in the exon 17 classic, as well as some patients with exon 12. The main methods of treatment used in patients were erythrocytapheresis IE, hydroxyurea, interferon alpha (α-IFN), both in mono and in combination. The results of treatment were obtained the following responses to therapy - complete hematological response partial hematological response and lack of response. Depending on the type of therapy the response rate was different. Also analyzed was the frequency of thrombosis in patients with PV. In the analyzed group were registered deaths and the phase postpolyciythe of myelofibrosis. In the result of the analysis conclusions are made about the results and possible prospects in treatment of PV.