title: [True polycythemia: current views of pathogenesis, diagnostics and treatment].
abstract: Current concepts of true polycythemia are reviewed. Results of numerous epidemiological studies on the prevalence of this form of myeloproliferative neoplasms are presented with special reference to recent findings concerning its pathogenesis and the role of JAK2V617F mutation that occurs in the majority of patients. The clinical picture and new diagnostic criteria are discussed The data on the incidence and prevalence of the disease and its complications are considered including venous and/or arterial thrombosis, transformation into post-polycythemic myelofibrosis and acute myeloleucosis. An algorithm for the treatment of patients with erythremia is proposed along with recommendations on the use of aspirin, hydroxyurea, alpha-interpheron, and imatinib. The prospects for clinical application of selective JAK2 inhibitors are discussed.
authors.forename: N T
authors.initials: NT
authors.lastname: Vatutin
authors.forename: G G
authors.initials: GG
authors.lastname: Taradin
authors.forename: T D
authors.initials: TD
authors.lastname: Bakhteeva
authors.forename: N V
authors.initials: NV
authors.lastname: Kalinkina
authors.forename: E V
authors.initials: EV
authors.lastname: Skliannaia
mesh_terms: D000367:Age Factors; D000465:Algorithms; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001241:Aspirin / Q000627:therapeutic use*; D001549:Benzamides; D003131:Combined Modality Therapy; D006400:Hematocrit; D006438:Hemodilution / Q000379:methods*; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D000068877:Imatinib Mesylate; D053614:Janus Kinase 2 / Q000037:antagonists & inhibitors / Q000235:genetics*; D015470:Leukemia, Myeloid, Acute / Q000209:etiology; D009154:Mutation; D010879:Piperazines / Q000627:therapeutic use*; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D011087:Polycythemia Vera / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000378:metabolism / Q000503:physiopathology / Q000628:therapy; D015995:Prevalence; D055728:Primary Myelofibrosis / Q000209:etiology; D011743:Pyrimidines / Q000627:therapeutic use*; D016032:Randomized Controlled Trials as Topic; D018570:Risk Assessment; D013927:Thrombosis / Q000209:etiology / Q000517:prevention & control; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D001549:Benzamides; D010879:Piperazines; D010975:Platelet Aggregation Inhibitors; D011743:Pyrimidines; D000068877:Imatinib Mesylate; D053614:Janus Kinase 2; D001241:Aspirin; D006918:Hydroxyurea