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Information × Registration Number 0217U004340, 0114U002849 , R & D reports Title Molecular-genetic factors of thrombosis in patients with Ph-negative myeloproliferative neoplasia who suffered from the Chernobyl accident popup.stage_title Head Klymenko Serhij Victorovych, Доктор медичних наук Registration Date 31-01-2017 Organization State institution "National research centеr for radiation medicine of the National academy of medical sciences of Ukraine " popup.description2 The object of study - general clinical, laboratory hematological, molecular-genetic characteristics of polycythemia veta (PV) , essential thrombocythemia (ET), idiopathic myelofibrosis (IMP) and myeloid neoplasms with eosinophilia (MNE) patients, including those, who were affected by the accident at the Chernobyl nuclear power Plant (CNPP). The purpose - to determine the value of mutations of the gene PDGFRA, PDGFRB, FGFR1, mutations JAK2V617F, allelic polymorphism of the coagulation factor II and V gene in the formation of the risk of thrombosis in Ph-negative MPN patients, who were affected by the Chernobyl accident, and justify the treatment algorithm on the based of the genetically thrombophilia. Methods - general clinical, molecular-genetic, cytogenetic statistics. The molecular-genetic and general-clinical characteristics of 78 and 429 patients with radiation-associated spontaneous MPN, MNE respectively were analyzed. It is proved, that the cardiac risk factors (CRF), hematocrit more than 55 % and more than 13.2 th/l leukocytes is advisable for the taking into account for the prediction of thrombosis in spontaneous and radiation-associated PV patients younger than 60 years without the vascular events history at the time of verification of the diagnosis (area under the ROC-curve (AUC) AUC = 0.99 and = 0.89 respectively). The criterion "platelets less than 440,0 th/l", despite of the fact, that it was only the trigger of thrombosis at radiation-associated PV in patients with a priori the high risk of their development, is not required for the taking into account for the assessing of the likelihood of vascular events. However, the determined the intensification of the contribution of the factor "leukocytes more than 13,2 th/l” to the dispersion of thrombotic status of radiation-associated PV patients in case of the presence of the indicator "platelets less than 440,0 th/l". JAK2V617F-positive mutation status and the amount of leukocytes more than 10,0 th/l for the prediction of thrombosis is recommended in patients with spontaneous and radiation-associated ET younger than 60 years without vascular events in the history at the time of verification of the diagnosis (AUC = 0.98 and AUC = 1.00 respectively). The carrying of G1691A allele of the coagulation factor V gene or the G20210A alleles of the coagulation factor II gene for the predicting of the likelihood of thrombosis should be considered in patients with spontaneous IMF without CRF (AUC = 0.92). At the radiation-associated IMF, due the lack of the leveling or potentiating interaction the hereditary thrombophilia markers with the CRF and age older than 60 years in relation to the protrombogenic influence, these general clinical and molecular-genetic indicators are recommended to use for the predicting of the development of vascular events a total cohort of patients each separately (AUC = 0.98). It is also confirmed, that in Ukraine, the frequency of the restructuring PDGFRA gene (5.20 % persons) and clinical and hematological characteristics of spontaneous and radiation-associated MNE patients corresponds to the one, which is defined in the other studies. This makes it possible to use the generally accepted criterion of the verification of MNE in patients with a history of radiation. The use of the recommendations for the diagnosis of MNE and the algorithms of the evaluation of an occurrence of vascular complications in Ph-negative MPN patients, affected by the Chernobyl accident, which were developed with the inclusion of molecular-genetic, cytogenetic characteristics, allows the use of the WHO 2016 classification and to extend the disease period without thromboses in Ukraine respectively. Product Description popup.authors Вербиленко Роксана Миколаївна Веропотвелян Миколо Петрович Клименко Сергій Вікторович Кравченко Сергій Миколайович Міщенюк Ольга Юріївна Неумержицька Любов Володимирівна Полубень Лариса Олександрівна Шкарупа Володимир Миколайович popup.nrat_date 2020-04-02 Close