Efficiency of anticoagulant protein C system in patients with Primary Myelofibrosis
ISTH Academy. Matvienko O. Jul 9, 2019; 264405; PB1214 Topic: Protein C/S Pathway
Mrs. Olesia Matvienko
Mrs. Olesia Matvienko
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Efficiency of Anticoagulant Protein C System in Patients with Primary Myelofibrosis

O. Matvienko, N. Silina, N. Korsakova, V. Kobilyanskaya, E. Efremova, M. Fominykh, L. Papayan, O. Golovina, V. Shuvaev, S. Voloshin
Russian Research Institute of Haematology and Transfusiology, Saint Petersburg, Russian Federation

Main Topic: Coagulation and Anticoagulation
Category: Protein C/S Pathway

Background: Primary Myelofibrosis (PMF) is philadelphia-negative myeloproliferative neoplasm with unique symptoms caused by bone marrow fibrosis. Imbalance of coagulation system in PMF patients leads to complications such as thrombosis and bleedings. Known risk factors of thrombotic complications are age over 60, thrombocitosis, leycocitosis and presence of JAK2V617F mutation. Ordinary coagulation tests of PMF patients frequently demonstrates multidirectional variations. Evaluating of anticoagulant system efficiency can be informative for thrombotic risk assessment.
Aims: To estimate the efficiency of protein C anticoagulant system in PMF patients.
Methods: The study included 30 PMF patients and 21 healthy controls. Thrombin generation was assessed by CAT according to Hemker et al. Measure was conducted in platelet poor plasma with or without presence of thrombomodulin (ТМ). The following parameters were determined: endogenous thrombin potential (ETP, nM*min), peak thrombin (Peak, nM). Sensitivity ETP and Peak for TM were calculated as percent of decreasing of these parameters after adding TM (S ETP, % and S Peak, % respectively). Reduction of sensitivity for TM indicates dysfunction of anticoagulant protein C system. Activity of protein C and S (%) was measured with 'ACL ELIT PRO' coagulometer, Instrumentation Laboratory, USA.
STATISTICA 6.0 package was used. Results are presented as median with 95% confidence intervals, p< 0.05 was considered statistically significant (*).
Results: Sensitivity ETP and Peak for TM, activity of protein C and S are presented in table.
Sensitivity ETP and Peak for TM was significant lower in patients as well as activity of protein S. A direct corelation was found between S ETP and activity of protein S (r =.0,44907).
Conclusions: The efficiency of protein C anticoagulant system is depressed in PMF patients. It may be connected with decrease of protein S activity. The disability of anticoagulant protein C system can lead to offset haemostatic balance to hypercoagulability and it may be cause of thrombotic complication.

Parameters Patients (n=30) Controls (n=21)
S ETP (%) 36,1 (4,63-62,0) * 51,53 (22,49-65,49)
S Peak (%) 24,0 (0,87-51,0) * 35,49 (14,56-53,78)
Activity of protein C (%) 95,5(48,01-141,0) 96,95 (74,8-127,7)
Activity of protein S (%) 78,0(49,2-130,1) * 123,6 (112,8-142,5)

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