title: The contribution of hereditary thrombophilia to increasing the frequency of thrombosis in patients with Ph negative myeloproliferative neoplasms, including the victims from the Chornobyl accident.
abstract: OBJECTIVE
  The definition of a contribution of the carriage of the G1691A allele of thecoagulation factor V gene and the G20210A allele of the coagulation factor II gene in the development of thrombosis in Ph negative myeloprolifer ative neoplasms (MPN) patients, who were irradiated in the dose range 0,001 0,99 Gy and who were not.
  
  
  METHODS
  The clinical and molecular genetic characteristics of patients with radiation associated and spontaneous polycythemia vera (PV), essential trombotsytemiya (ET) and primary myelofibrosis (PMF) were ana lyzed. The group of radiation associated PV, ET and PMF represented by 35, 10 and 22 patients respectively, and the cohort of spontaneous PV, ET and PMF - 149, 111 and 78 patients respectively.
  
  
  CONCLUSIONS
  The carriage of any of the two molecular genetic markers of hereditary thrombophilia at spontaneous PMF increases the frequency (3 of 6 vs 8 of 72; p = 0.033) and risk (RR = 6.09; 95 % CI = 1.40-26.43) of thrombosis. The presence of the G1691A allele of the proaccelerin gene in patients with PMF, who were not exposed to ionizing radiation, causes increase the likelihood of venous thrombosis at 10.14 times (95 % CI = 1.67-61.33). At spontaneous and radiation associated Ph negative MPN (in individuals exposed to doses in the range 0,001-0,99 Gy), the higher rate of the occurrence of venous, arterial and any thrombosis was observed in carriers of the G1691A allele the coagulation factor V gene, than in those, whose have the wild type allele. In particular, the G1691A allele of the proaccelerin gene carriers, that are belonged to the group of patients with radiation associated PV, have at 33.33 person years bigger rate of any thrombosis (95 % CI = 0.22-100.00, p = 0.048) and venous vascular events (95 % CI = 12.50-50.00; p = 0.003).In PMF patients with a radiation anamnesis were found the difference (20.00 person years; 95 % CI = 1.51-50.00, p = 0.035) between the ratio of any thrombosis and arterial vascular events, which was calculated for the G1691A allele of the proaccelerin gene and for those, who have the wild type allele. The carriers of the G20210A nucleotide variant of the coagulation factor II gene with spontaneous ET and PMF, compared with patients with the wild type allele, have a higher rate of venous thrombosis per 100 patient years.
authors.affiliation: State Institution National Research Center for Radiation Medicine of the National Academy of Medical Sciences of Ukraine, 53 Melnykova Street, Kyiv, 04050, Ukraine.
authors.forename: O Y
authors.initials: OY
authors.lastname: Mishcheniuk
authors.affiliation: National Pirogov Memorial Medical University, Pirogov street, 56, Vinnytsia, 21000,Ukraine.
authors.forename: V M
authors.initials: VM
authors.lastname: Shkarupa
authors.affiliation: Scientific and Practical Center of Preventive and Clinical Medicine, Verhnya street, 5, Kiev, 01014, Ukraine.
authors.forename: O M
authors.initials: OM
authors.lastname: Kostukevich
authors.affiliation: State Institution National Research Center for Radiation Medicine of the National Academy of Medical Sciences of Ukraine, 53 Melnykova Street, Kyiv, 04050, Ukraine.
authors.forename: L V
authors.initials: LV
authors.lastname: Neumerzhitcka
authors.affiliation: State Institution National Research Center for Radiation Medicine of the National Academy of Medical Sciences of Ukraine, 53 Melnykova Street, Kyiv, 04050, Ukraine.
authors.forename: S M
authors.initials: SM
authors.lastname: Kravchenko
authors.affiliation: State Institution National Research Center for Radiation Medicine of the National Academy of Medical Sciences of Ukraine, 53 Melnykova Street, Kyiv, 04050, Ukraine.
authors.forename: S V
authors.initials: SV
authors.lastname: Klymenko
mesh_terms: D047852:Chernobyl Nuclear Accident; D005165:Factor V; D006801:Humans; D009196:Myeloproliferative Disorders; D019851:Thrombophilia; D013927:Thrombosis
chemical_list: D005165:Factor V
keywords: Ph-negative myeloproliferative neoplasm; inherited thrombophilia; thrombosis