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THROMBOSIS IN HEMATOLOGICAL PRACTICE
Tursunova Nigora Abduvalievna, Makhmudova Aziza Djumanovna, Shadibekova Oksana Borisovna, Berger Inna Viktorovna, Research Scientific Institute of Hematology and Blood Transfusion MoH of Uzbekistan Republic Uzbekistan, Tashkent E-mail: innaberger@mail.ru
THROMBOSIS IN HEMATOLOGICAL PRACTICE
Abstract: Hemostasis disorders are a frequent pathological condition and are characterized by a high potential hazard. Hemostasiopathies can manifest themselves as independent syndromes, as well as secondary hemorrhagic and thrombotic complications of a large number of other diseases: infectious, cardiovascular, blood diseases, etc. The problem ofprevention and therapy of thromboembolism, ischemia and infarction of organs continues to occupy a central place in modern clinical medicine, as these types of pathology are very frequent and dominate among the causes of sudden death of people and their early disability.
Keywords: thrombophilia, hemostasiology, thromboprophylaxis, hypercoagulation, thrombogenic risk.
Thrombosis of arterial and venous vessels play a big role in quent reception of indirect anticoagulants under the con-the pathogenesis of the most frequent and dangerous human trol of INR. At parents the anamnesis on vascular accidents diseases. The mortality from ischemic heart disease (IHD) is negative. According to the coagulogram data, high levels
and ischemic brain disease (IBM) is 40-45%. The frequency of thrombosis of the arteries of the heart with myocardial infarction is 85-70%. Thrombosis of the brain vessels determine the development of strokes in 75-80% of cases. Pulmonary arterial thromboembolism (PE) is found in 16% of all autopsies, and is diagnosed during life only in 30% of cases [3]. The presence of thrombophilia is associated with an increased risk of complications of pregnancy.
There are genetic, acquired and iatrogenic thrombophilia.
There are many mutations of hemostasis genes, and a number of mutations have a high prothrombogenic potential and can often lead to thrombotic complications [1]. Among these mutations, the mutation of the factor V gene (1691G \ A Leiden), mutation of the prothrombin gene FII (20210G \ A) is especially significant. Patients with homozygous mutations even in one of the genes rarely survive to the age of over 30 years, the combination of heterozygous forms of these mutations also significantly increases the likelihood of thrombosis of various localizations [1, 2].
All this is the basis for an active search for the causes of intravascular thrombosis.
Clinical cases: Example No. 1 Patient E., 20 years old. Turned to the clinic in 2015 in the direction of the vascular surgeon, to which he addressed with complaints of pain and swelling of the left lower limb, weakness. Acute venous thrombosis of deep veins of the tibia was revealed on the left. Treatment received low-molecular heparins with the subse-
of thrombinemia markers (by the amount of soluble fibrin monomer complexes and D-dimer) were found, increasing platelet aggregation when evaluating their function with ADP and collagen; slowing the time ofXIIa-dependent fibrinolysis, increasing the activity of factor VIII. No genetic examination was conducted.
A study was recommended for the presence of genetic markers of thrombogenic risk: Conducting courses of throm-botic prophylaxis aimed at correcting hyperaggregation syndrome (antiplatelet agents after sensitivity was established to them, for a long time), thrombinemia (low molecular weight heparins in preventive doses); Dynamic control of thrombotic readiness markers (2 times a year) and, if necessary, a repeat course of thromboprophylaxis. Result. Over the past 2 years of follow-up, following the recommendations, the patient did not have a second thrombosis.
Example No. 2 Patient H., 22, was consulted in December 2017 in the subacute period after acute deep vein thrombosis of the right lower limb. Varicose disease of the lower extremities was also revealed, the patient received direct anticoagulants, then was transferred to direct inhibitors of the X factor. In the laboratory study of hemostasis after the establishment of thrombosis, a high level of markers of thrombinemia (soluble fibrin and D-dimers) was found. The conclusion. Thrombophilia is a condition of thrombotic readiness caused by an increase in the level of markers of thrombinemia (soluble fibrin and D-dimers). Recommended. The use of sodium enoxaparin
Section 11. Medicine
in a therapeutic dose (subcutaneously, 1 mg / kg body weight every 12 hours). Determination of the level of homocysteine, aggregation function of thrombocytes. Monitoring the level of thrombinemia markers and duplex scanning of the veins of the lower extremities - after 1 and 3 months.
It can be noted that the above, diverse clinical examples unite the influence of a wide range of permanent or temporary factors of thrombogenic risk. However, it is the presence or absence of thrombotic readiness in patients, determined by objective laboratory data, which gives grounds for the use of antithrombotic agents.
According to literature data 1. Most people who are carriers of permanent or temporary risk factors for thrombosis do not suffer from thrombosis throughout life, although they are likely to develop this pathology [1]. Nevertheless, the presence of factors of thrombogenic risk is often equated with thrombophilia, which leads to overdiagnosis of thrombophilia. Obviously, thrombophilia can only be indicated for those conditions that have manifested themselves as repeated thromboses or fetal loss syndrome in an individual anamnesis.
2. Between the presence of factors of thrombogenic risk and thrombosis, the state of prethrombosis is often identified, often described as "hypercoagulable syndrome / condition". It is more justified to use the alternative concept of "thrombotic readiness state" capable of combining a laboratory-determined tendency to hypercoagulability and clinical signs of prethrombosis. It is the implementation ofthis readiness with the remaining factors of thrombogenic risk and their combination with a high probability is capable of manifesting a vascular catastrophe.
3. The state of thrombotic readiness precedes thrombosis, and also accompanies it. To identify this condition, it is necessary to take into account the activity of platelets and the level of markers of activation of the coagulation unit of hemostasis, primarily D-dimers.
Patients should be examined for the possible presence of thrombophilia:
- Idiopathic thrombosis; recurrent thrombosis in persons younger than 50; Thromboembolic complications in close relatives in the anamnesis;
- Thrombosis of unusual localization (Badd-Kiari syndrome, mesenteric thrombosis, central vein thrombosis); thrombosis on the background of COC and HRT; Ovarian hyperstimulation syndrome with IVF; obstetric complications in the anamnesis;
- Skin necrosis on the background of taking oral anticoagulants.
Depending on the situation and indices of hemostasis, it is recommended:
Determination of the level of natural anticoagulants (AT III, protein C, protein S), level of clotting factors (factor VIII, factor IX, von Willebrand factor), plasminogen level (fibrinolysis activator), homocysteine level, complex aggre-gationgram (spontaneous aggregation and aggregation with inducers), exclusion of APS (lupus anticoagulant, antibodies to cardiolipins, antibodies to phospholipids, antibodies to ^-glycoprotein 1, antibodies to annexin V), hemostasis mutations.
References:
1. Makatsariya A. D., Bitsadze V. O. Thrombophilia and antithrombotic therapy in obstetric practice.- M., "Triad-X",- 2003.
2. Beral V., Banks E., Reeves G. Evidence from randomised trials on the long-term effects of hormone replacement therapy // Lancet.- 2002.- Vol. 360.- No. 9337.- P. 942-944.
3. Lowe G. D. Circulating inflammatory markers of cardiovascular and non-cardiovascular disease // J of Thromb and Hae-most.- 2005.- Vol. 3.- No. 8.- P. 1618-1627.
