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 myeloproliferative neoplasms (MPN) patients, who were irradiated in the dose range 0,001-0,99 Gy and who were not. Materials and 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 analyzed. 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. Results and 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 G1691Aa[[ele of the proaccelerin genein 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.