title: Diagnosis and treatment of polycythemia vera: Brazilian experience from a single institution.
abstract: OBJECTIVE
  Polycythemia vera (PV) is a chronic myeloproliferative disorder characterized by predominant proliferation of erythroid precursors. Few data are available concerning Brazilian patients with this condition. The aim of this study was to describe clinical and demographic characteristics of PV patients at diagnosis and analyze their long-term outcomes.
  
  
  METHODS
  Retrospective study at the Division of Hematology, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo.
  
  
  METHODS
  All consecutive patients with PV diagnosed according to World Health Organization criteria were eligible for this study. Clinical and demographic characteristics, thrombotic events, transformation to acute leukemia, myelofibrosis and survival were evaluated.
  
  
  RESULTS
  Sixty-six patients were evaluated. Thirty-six (54.5%) were females, with a median age at diagnosis of 61 years. At diagnosis, the median hemoglobin concentration was 18.8 mg/dl and the median platelet count was 593,000/mm(3). Fifty-eight patients (88.0%) were treated with hydroxyurea with or without phlebotomy. During a median follow-up of 77 months, 22 patients (33.3%) had new thrombotic events, mainly of arterial type. The overall incidence of leukemia and myelofibrosis was 0.42% per patient-year and 1.06% per patient-year, respectively. Median overall survival was not reached and the seven-year survival rate was 77.8%.
  
  
  CONCLUSIONS
  The PV patients described here had long survival and arterial thrombotic events were the most important and common complication among this population.
authors.affiliation: Division of Hematology, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.
authors.forename: Camila da Cruz Gouveia
authors.initials: Cda C
authors.lastname: Linardi
authors.forename: Luís Fernando
authors.initials: LF
authors.lastname: Pracchia
authors.forename: Valeria
authors.initials: V
authors.lastname: Buccheri
mesh_terms: D000328:Adult; D001938:Brazil / Q000453:epidemiology; D004812:Epidemiologic Methods; D005260:Female; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D007938:Leukemia / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010976:Platelet Count; D011087:Polycythemia Vera / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D013927:Thrombosis / Q000150:complications; D016896:Treatment Outcome
chemical_list: D006454:Hemoglobins