title: Specifics of the course and the clinical pictures of essential thrombocythemia depending on the JAK2V617F status of the patients.
abstract: OBJECTIVE
  The purpose of the study was to analyze the specific features of the relationship between the JAK2 V617Fmutation and clinical presentation, and risks of thrombosis in patients with spontaneous and radiation associated essential trombocitemia (ET).
  
  
  METHODS
  Clinical, laboratory hematological, molecular genetic characteristics of ET patients were analyzed. The group of patients with radiation associated ET was represented by 10 individuals and the cohort of spontaneous ET represented by 111 persons.
  
  
  RESULTS
  The JAK2V617F positive mutation status in patients with spontaneous ET is the predictor of the increase of distribution of thrombosis (23.0 % vs. 6.0 %), risk of its development (relative risk = 1.5; 95 % confidence interval = 1.3-1.8), and the decrease of survival without thrombosis. It has been proved that presence of the JAK2V617F mutation increases the probability of venous thrombosis (relative risk = 1.5; 95 % confidence interval = 1.30-1.7) in spontaneous ET patients. The use of the JAK2V617F mutation status of patients with spontaneous and radiation associated ET (AUC = 0.62 and AUC = 0.68 respectively) complies with the average power marker for the prediction of thrombosis. The carriage of the JAK2V617F mutation in patients with spontaneous and radiation associated ET is characterized by a higher (93.9 % and 100 % accordingly) indicator of predictive value of a negative result to deter mine of the risk of thrombosis. ET patients with the JAK2V617F mutation were found to have a higher average value of erythrocytes, hemoglobin, mean corpuscular volume and lower erythrocyte sedimentation rate in their peripher al blood, when compared to those without the mutation. Trepanobioptat samples of JAK2V617F positive ET patients exhibited a prevalence of moderate hypercellularity of bone marrow, large and giant forms of megakaryocytes, reti culin fibrosis and microcirculation disorders compared to individuals without the mutation. A greater number of average quantities of leukocytes in JAK2V617F positive patients with radiation associated ET were found compared to JAK2V617F negative persons.
authors.affiliation: State Institution National Research Center for Radiation Medicine of National Academy of Medical Sciences of Ukraine (NRCRM), Melnikova str., 53, Kyiv, 04050, Ukraine.
authors.forename: O Y
authors.initials: OY
authors.lastname: Mishcheniuk
authors.affiliation: State Institution Scientific and Practical Center of Preventive and Clinical Medicine of the State Administration, Verkhnia str., 5, Kyiv, 01014, Ukraine.
authors.forename: O M
authors.initials: OM
authors.lastname: Kostukevich
authors.affiliation: State Institution National Research Center for Radiation Medicine of National Academy of Medical Sciences of Ukraine (NRCRM), Melnikova str., 53, Kyiv, 04050, Ukraine.
authors.forename: I V
authors.initials: IV
authors.lastname: Dmytrenko
authors.affiliation: State Institution National Research Center for Radiation Medicine of National Academy of Medical Sciences of Ukraine (NRCRM), Melnikova str., 53, Kyiv, 04050, Ukraine.
authors.forename: S V
authors.initials: SV
authors.lastname: Klymenko
keywords: JAK2V617F mutation; clinical laboratorial characteristics; essential trombocitemia; thrombosis