TY - JOUR

T1 - HEMOSTATIC MARKERS OF HYPERCOAGULABILITY IN PRIMARY MYELOFIBROSIS PATIENTS, RELATION WITH JAK2V617F ALLELE BURDEN

AU - Korsakova, N.

AU - Silina, N.

AU - Efremova, E.

AU - Fominykh, M.

AU - Kobilyanskaya, V.

AU - Golovina, O.

AU - Matvienko, O.

AU - Polushkina, L.

AU - Martynkevich, I.

AU - Shuvaev, V.

AU - Voloshin, S.

AU - Papayan, L.

PY - 2019

Y1 - 2019

N2 - Background: Thrombotic complications contribute significantly to morbidity and mortality in Ph-negative myeloproliferative neoplasms, including primary myelofibrosis (PMF). The majority of PMF patients have JAK2V617F mutation that is associated with increased thrombotic risk, CALR gene mutations are also common in PMF. Clonal myeloproliferation in PMF followed by secondary inflammation with pathologic cytokine production induce endothelium, leukocytes and platelets activation resulting in coagulation activation. Natural anticoagulants protein C (PC) and protein S (PS) deficiencies with activated protein C resistance development are supposed among the crucial procoagulant features in Polycythemia Vera and Essential Thrombocythemia, while the data on hemostatic abnormalities in PMF and their relationship with JAK2V617F burden is scarce. Aims: To evaluate the hemostatic parameters in PMF patients, predisposing high thrombosis risk, and their relationship with JAK2V617F presence and allele burden. Methods: The study included 36 PMF patients (age 30-86 years, median 60 years). Somatic mutations (JAK2V617F and CALR) presence and JAK2V617F allele burden were assessed using allele specific polymerase-chain reaction. The control group consisted of 68 healthy persons (30-73 years, median 46 years). Coagulation assays (factors VIII, V, von Willebrand (VWF), antithrombin and PC activities, fibrinogen concentration, VWF antigen and free PS level) were performed by standard clotting, chromogenic and immunoturbidimetric techniques. STATISTICA 6.0 was used for data analysis. The results were presented as median with 95% confidence interval (CI). Mann-Whitney and Spearman rank tests were applied, the differences considered statistically significant with p 

AB - Background: Thrombotic complications contribute significantly to morbidity and mortality in Ph-negative myeloproliferative neoplasms, including primary myelofibrosis (PMF). The majority of PMF patients have JAK2V617F mutation that is associated with increased thrombotic risk, CALR gene mutations are also common in PMF. Clonal myeloproliferation in PMF followed by secondary inflammation with pathologic cytokine production induce endothelium, leukocytes and platelets activation resulting in coagulation activation. Natural anticoagulants protein C (PC) and protein S (PS) deficiencies with activated protein C resistance development are supposed among the crucial procoagulant features in Polycythemia Vera and Essential Thrombocythemia, while the data on hemostatic abnormalities in PMF and their relationship with JAK2V617F burden is scarce. Aims: To evaluate the hemostatic parameters in PMF patients, predisposing high thrombosis risk, and their relationship with JAK2V617F presence and allele burden. Methods: The study included 36 PMF patients (age 30-86 years, median 60 years). Somatic mutations (JAK2V617F and CALR) presence and JAK2V617F allele burden were assessed using allele specific polymerase-chain reaction. The control group consisted of 68 healthy persons (30-73 years, median 46 years). Coagulation assays (factors VIII, V, von Willebrand (VWF), antithrombin and PC activities, fibrinogen concentration, VWF antigen and free PS level) were performed by standard clotting, chromogenic and immunoturbidimetric techniques. STATISTICA 6.0 was used for data analysis. The results were presented as median with 95% confidence interval (CI). Mann-Whitney and Spearman rank tests were applied, the differences considered statistically significant with p 

M3 - Article

VL - 3

JO - HemaSphere

JF - HemaSphere

SN - 2572-9241

IS - S1

M1 - PB2206

ER -