COAGULATION HEMOSTASIS: MODERN VIEW, PATHOLOGY, DIAGNOSTICS
https://doi.org/10.57256/2949-0715-2025-4-2-32-52
Abstract
Relevance. The hemostasis system is a complex multi-level mechanism that, on the one hand, prevents blood loss when a vessel is damaged, and on the other hand, maintains the blood in a liquid state, preventing excessive thrombi formation. It is implemented with the participation of blood cells, endothelial cells and other cells, as well as several sequentially and mutually activated enzyme systems. There are two types of hemostasis: primary and secondary. As a result of activation of primary hemostasis, a platelet thrombus is formed, secondary hemostasis results in the formation of a fibrin clot. Hemostasis disorders lead to either bleeding or thrombosis, which is one of the most important causes of mortality and morbidity in the world.
Aim. To consider modern concepts of the mechanisms of fibrin clot formation in norm and pathology, to provide screening tests for evaluation the pathology of coagulation hemostasis.
Results. The review examines the sequence of activation of coagulation hemostasis factors from the standpoint of cellular theory. A description and characteristics of most of the participants in the coagulation hemostasis process are separately provided. The most common causes, mechanisms and manifestations of hypo- and hypercoagulation are described. At the end, the most common screening tests for analyzing the state of coagulation hemostasis are described.
Conclusion. According to the cellular model, hemostasis occurs in four overlapping phases (initiation, amplification, propagation, and termination) and occurs on different cell surfaces. Specifically, coagulation is initiated in cells expressing tissue factor, amplifies and propagates on the phospholipid surface of fully activated platelets. Hemostasis involves complex biochemical mechanisms and interactions between the vascular endothelium, coagulation factors, and cellular (mainly platelets) blood components.
About the Authors
Lyudmila O. GutsolRussian Federation
Cand. Sci. (Biol.), Associate Professor, Associate Professor of the Department of Pathological Physiology and Clinical Laboratory Diagnostics
Irina E. Egorova
Russian Federation
Cand. Sci. (Med.), associate professor, associate professor of the Department of Chemistry and Biochemistry
Igor Zh. Seminsky
Russian Federation
Dr. Sci. (Med.), Professor, Head of the Department of Pathological Physiology and Clinical Laboratory Diagnostics
Evgenia V. Guzovskaiia
Russian Federation
Cand. Sci. (Med.), associate professor of the Department of Pathological Physiology and Clinical Laboratory Diagnostics
Svetlana N. N. Serebrennikova
Russian Federation
Cand. Sci. (Med.), associate professor of the Department of Pathological Physiology and Clinical Laboratory Diagnostics
Lyudmila A. Dmitrieva
Russian Federation
Cand. Sci. (Med.), Head of the Laboratory of Clinical Diagnostics
References
1. Леонтьева Н.В. Характеристика факторов свертывания крови. Актуальные проблемы теоретической и клинической медицины. 2019; 3(25):9-13 [Leontyeva N.V. Characteristics of blood coagulation factors. Actual problems of theoretical and clinical medicine. 2019, 3(25):9-13 (In Russian)]. URL: https://kazrosmedjournal.krmu.edu.kz/jour/article/view/191 [дата доступа: 08.05.2025]
2. Кузник Б.И. Клеточные и молекулярные механизмы регуляции системы гемостаза в норме и патологии. Чита:Экспресс-издательство; 2010:832 [Kuznik B.I. Cellular and molecular mechanisms of regulation of the hemostasis system in norm and pathology. Chita:Еxpress publishing house; 2010:832 (In Russian)]
3. Леонтьева Н.В. Каскадная модель свертывания крови. Актуальные проблемы теоретической и клинической медицины. 2019;3(25):14-18. [Leontyeva N.V. Cascade model of blood coagulation. Actual problems of theoretical and clinical medicine. 2019;3(25):14-18 (In Russian)]. URL: https://kazrosmedjournal.krmu.edu.kz/jour/article/view/192/190?locale=ru_RU [дата доступа: 08.05.2025]
4. Счастливцев И.В., Лобастов К.В., Цаплин С.Н. и др. Современный взгляд на систему гемостаза: клеточная теория. Медицинский совет. 2019; 16:72-77 [Schastlivtsev I.V., Lobastov K.V., Tsaplin S.N. et al. A modern view of the hemostasis system: cellular theory. Medical Council. 2019, 16:72-77 (In Russian)]. https://doi.org/10.21518/2079-701X-2019-16-72-77
5. Butenas S., Orfeo T., Mann K.G. Tissue factor in coagulation: Which? Where? When? Arterioscler Thromb Vasc Biol. 2009;29(12):1989-1996. https://doi.org/10.1161/ATVBAHA.108.177402
6. Long A.T., Kenne E., Jung R., Fuchs T.A., Renné T. Contact system revisited: an interface between inflammation, coagulation, and innate immunity/ Jоurnal thrombosis and haemostasis. 2015;14(3):427-437. https://doi.org/10.1111/jth.13235
7. Versteeg H.H., Heemskerk Johan W.M., Levi M., Reitsma P.H. New Fundamentals in Hemostasis. Рhysiological Reviews. 2013;93(1):327–358. https://doi.org/10.1152/physrev.00016.2011
8. Шашок Л.В., Кабаева Е.Н., Цвирко Д.Г. Дефицит FXII фактора свертывания (фактора Хагемана) – редкая коагулопатия или частая лабораторная находка? Лабораторная диагностика Восточная Европа. 2023;12(4):551-557 [Shashok L.V., Kabaeva E.N., Tsvirko D.G. Deficiency of FXII Coagulation Factor (Hageman Factor) is a Rare Coagulopathy or a Common Laboratory Finding? Laboratory Diagnostics Eastern Europe. 2023;12(4):551-557. (In Russian)] https://doi.org/10.34883/PI.2023.12.4.006
9. Maat S. Maas C. Factor XII: form determines function. Journal of Thrombosis and Haemostasis. 2016;14(18):1498-1506. https://doi.org/10.1111/jth.13383
10. Яровая Г.А., Блохина Т.Б., Нешкова Е.А. Контактная активация протеолитических систем плазмы крови. Новые концепции о механизмах активации и биорегулирующих функциях. Лабораторная медицина. 2008;9:19-26 [Yarovaya G.A., Blokhina T.B., Neshkova E.A. Contact activation of proteolytic systems of blood plasma. New concepts on mechanisms of activation and bioregulatory functions. Laboratory Medicine. 2008,9:19-26 (In Russian)]. https://doi.org/10.17116/oftalma2016132488-93
11. Яковлева Е.В., Зозуля Н.И. Физиологическая и патологическая роль фактора свертывания крови XII. Гематология и трансфузиология. 2022,67(4):570-578 [Yakovleva E.V., Zozulya N.I. Physiological and pathological role of factor XII. Russian journal of hematology and transfusiology. 2022;67(4):570-578 (In Russian)]. https://doi.org/10.35754/0234-5730-2022-67-4-570-578
12. Gale A.J. Continuing education course №2: current understanding of hemostasis. Toxicol Pathol. 2011;39(1):273-80. https://doi: 10.1177/0192623310389474
13. Литвицкий П.Ф. Патология системы гемостаза. Вопросы современной педиатрии. 2014;13(2):65-76 [Litvitsky P.F. Pathology of the hemostasis system. Issues of modern pediatrics. 2014;13(2):65-76 (In Russian)]. https://doi.org/10.15690/vsp.v13i2.974
14. Гемофилия. Клинические рекомендации РФ, 2023. Утверждены Минздравом РФ. [Hemophilia. Clinical guidelines of the Russian Federation, 2023. Approved by the Ministry of Health of the Russian Federation (In Russian)]. URL: https://cr.minzdrav.gov.ru/recomend/127 [дата доступа: 08.05.2025]
15. Редкие коагулопатии: наследственный дефицит факторов свертывания крови II, VII, X. Клинические рекомендации. Утверждены Минздравом России, 2023 [Rare coagulopathies: hereditary deficiency of coagulation factors II, VII, X. Clinical guidelines. Approved by the Ministry of Health of the Russian Federation, 2023 (In Russian)]. URL: https://cr.minzdrav.gov.ru/recomend/149 [дата доступа: 08.05.2025].
16. Флоринский Д.Б., Жарков П.А. Редкие коагулопатии. Российский журнал детской гематологии и онкологии. 2020;7(3):54-63 [Florinsky D.B., Zharkov P.A. Rare coagulopathies. Russian Journal of Pediatric Hematology and Oncology. 2020;7(3):54-63 (In Russian)]. https://doi.org/10.21682/2311-1267-2020-7-3-54-63
17. Lippi G., Franchini M., Montagnana M., Favaloro E. J.. Inherited disorders of blood coagulation. Annals of Medicine. 2011;44(5):405–418. https://doi.org/10.3109/07853890.2011.576698
18. Palta S., Saroa R., Palta A. Overview of the coagulation system. Indian J Anaesth. 2014;58(5):515-523. https://doi.org/10.4103/0019-5049.144643
19. Albagoush S.A., Koya S., Chakraborty R.K., Schmidt A.E. Factor V Leiden Mutation. 2023 Apr 8. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025. URL: https://www.ncbi.nlm.nih.gov/books/NBK534802/ [дата доступа: 08.05.2025]
20. Костюченко Г.И. Гипергомоцистеинемия: клиническое значение, возрастные особенности, диагностика и коррекция. Клиническая геронтология. 2007;4:32-40 [Kostyuchenko G.I. Hyperhomocysteinemia: clinical significance, age-related features, diagnostics and correction. Clinical Gerontology. 2007;4:32-40 (In Russian)]. URL: https://cyberleninka.ru/article/n/gipergomotsisteinemiya-klinicheskoe-znachenie-vozrastnye-osobennosti-diagnostika-i-korrektsiya [дата доступа: 08.05.2025]
21. Dong-Feng Wu, Rui-Xing Yin, Jin-Long Deng, Homocysteine, hyperhomocysteinemia, and H-type hypertension, European Journal of Preventive Cardiology.2024 31(9):1092–1103. https://doi.org/10.1093/eurjpc/zwae022
22. Coppola A., Davi G., De Stefano V., [et al.] Homocysteine, coagulation, platelet function, and thrombosis. Semin Thromb Hemost. 2000;26(3):243-254. https://doi.org/ 10.1055/s-2000-8469
23. Воробьев А.И., Васильев С.А., Городецкий В.М. и др. Гиперкоагуляционный синдром: классификация, патогенез, диагностика, терапия. Гематология и трансфузиология. 2016;61(3):116-122 [Vorobiev A.I., Vasiliev S.A., Gorodetsky V.M. et al. Hypercoagulation syndrome: classification, pathogenesis, diagnostics, therapy. Hematology and transfusiology. 2016;61(3):116-122 (In Russian)]. https://doi.org/10.18821/0234-5730-2016-61-3-116-122
24. Бондаренко И.З. Ширшина И.А. Механизмы тромбообразования, ассоциированные с сахарным диабетом: что определяет прогноз интервенционного вмешательства. Сахарный диабет. 2013;16(3):58-63 [Bondarenko I.Z., Shirshina I.A. Mechanisms of thrombus formation associated with diabetes mellitus: what determines the prognosis of interventional intervention. Diabetes mellitus. 2013;16(3):58-63 (In Russian)]. https://doi.org/10.14341/2072-0351-95
25. Бернс А.С., Советников Е.Н., Чеботарева Н.В. и др. Оценка нарушений гемостаза с использованием теста тромбодинамики у больных хроническим гломерулонефритом с нефротическим синдромом. Терапевтический архив. 2022;94(6):738-742 [Burns A.S., Sovetnikov E.N., Chebotareva N.V., et al. Evaluation of hemostasis disorders using a thrombodynamics test in patients with chronic glomerulonephritis with nephrotic syndrome. Therapeutic archive. 2022;94(6):738–742(In Russian)]. https://doi.org/10.26442/00403660.2022.06.201558
26. Arreola-Diaz R., Majluf-Cruz A., Sanchez-Torres L., Hernandez-Juarez J. The Pathophysiology of The Antiphospholipid Syndrome: A Perspective From The Blood Coagulation System. Clinical and Applied Thrombosis/Hemostasis. 2022;28. https://doi.org/ 10.1177/10760296221088576
27. Кишкун А.А. Клиническая лабораторная диагностика. М.: ГЭОТАР-Медиа;2021:720 [Kishkun A.A. Clinical laboratory diagnostics. М.: GEOTAR-Media; 2021:720 (In Russian)] https://doi.org/10.33029/9704-6084-9-CLD1-2021-1-784
28. Вавилова Т.В., Варданян А.В., Самойленко В.В. и др. Интерпретация коагулограммы при нарушениях свертывания крови. М.:ГЭОТАР-Медиа; 2024:176 [Vavilova T.V., Vardanyan A.V., Samoylenko V.V. et al. Interpretation of coagulogram in blood clotting disorders. M.: GEOTAR-Media;2024:176 (In Russian)]. https://doi.org: 10.33029/9704-8111-0-ICD-2024-1-176.
29. Долгов В.В. Клиническая лабораторная диагностика. В 2 томах. Том 1. национальное руководство / Под ред. В. В. Долгова. Москва : ГЭОТАР-Медиа, 2013:928 [Dolgov V.V. Clinical laboratory diagnostics. In 2 volumes. Volume 1: national guidelines / Ed. by V.V. Dolgov. Moscow: GEOTAR-Media, 2013:928(In Russian)]. URL : https://www.rosmedlib.ru/book/ISBN9785970421291.html [дата доступа: 08.05.2025]
30. Система гемостаза. Теоретические основы и клиническая практика. Под ред. Рукавицына О.А., Игнатьева С.В. Москва : ГЭОТАР-Медиа;2024:944 [Hemostasis system. Theoretical foundations and clinical practice. Edited by Rukavitsyn O.A., Ignatiev S.V. Moscow: GEOTAR-Media; 2024:944 (In Russian)]. https://doi.org/10.33029/9704-8497-5-THS-2024-1-944
31. Ревматология : национальное руководство. Под ред. Е.Л. Насонова, В.А. Насоновой. М.: ГЭОТАР-Медиа; 2010:720. [Rheumatology: national guide. Ed. E.L. Nasonova, V.A. Nasonova. M.:GEOTAR-Media;2010:720 (In Russian)]. URL : https://www.rosmedlib.ru/book/ISBN9785970416501.html [дата доступа: 08.05.2025]
Review
For citations:
Gutsol L.O., Egorova I.E., Seminsky I.Zh., Guzovskaiia E.V., Serebrennikova S.N., Dmitrieva L. COAGULATION HEMOSTASIS: MODERN VIEW, PATHOLOGY, DIAGNOSTICS. Baikal Medical Journal. 2025;4(2):32-52. (In Russ.) https://doi.org/10.57256/2949-0715-2025-4-2-32-52