[Thrombophilia and thrombosis].

ABSTRACT

INTRODUCTION: Generally speaking, thrombophilia includes all congenital and acquired conditions associated with increased susceptibility to thrombosis. An impaired balance between stimulating and inhibitory components of the hemostasis system may result in thrombosis or hemorrhage (extreme disorders), while moderately impaired hemostasis induces thrombophilia. THROMBOPHILIA, PRETHROMBOTIC STATE AND THROMBOSIS A prethrombotic state is a condition of stimulated hemostasis system with tolerable intensity. When the activation level exceeds the capacity of inhibitory components, prethrombotic state advances to thrombosis. CLASSIFICATION OF THROMBOPHILIA Thrombophilia is classified as primary or hereditary and secondary or acquired. Hereditary or congenital thrombophilia is a condition of congenital mutation of one or more antithrombotic components. Acquired or secondary thrombophilia accompanies a variety of pathologic conditions and diseases. LABORATORY DIAGNOSIS OF THROMBOPHILIA Specific laboratory tests for congenital thrombophilia include assessment of the antigen component related to the activity of AT III, protein C, and protein S, evaluation of the resistance to the activated C protein, assessment of t-Pa and PAl-1 activity, as well as tests for hyperhomocysteinemia and prothrombin 20210 mutation. MANAGEMENT OF THROMBOPHILIA Treatment of patients with congenital AT III deficiency includes intravenous heparin and AT III concentrate (dosage 50 U/ kgbw). Patients with heterozygous protein C and S deficiency are treated by intravenous heparin and oral anticoagulant therapy. Regarding arterial thrombosis due to confirmed congenital hyperhomocysteinemia, it is treated the same as venous thrombosis.