Relevance. Essential thrombocytosis (ET) is a pathology of clonal hematopoietic stem cells, which is manifested by increased platelet formation. This pathology is quite rare and refers to myeloproliferative diseases. It is more common for women in two age groups: 50-70 y.o. and 20-30 y.o.
Objective. Evaluate diagnostic criteria, determine the risk of complications and, depending on this, a treatment plan for essential thrombocytosis.
Methods. Analysis of scientific publications in PubMed by keywords for the period 2006-2020. Case Comparison.
Results. The article discusses the pathogenesis, clinical picture, diagnostic criteria, determining the risk of complications and, depending on this, the treatment plan. ET is characterized with an increase in the number of megakaryocytes (MGKC) in the bone marrow (BM) and morphological changes in platelets, resulting in functional impairment of the latter. ET has long asymptomatic period. Manifestations of the disease are opposite: thrombosis and bleeding. Among multiple vessels that can be impaired, thrombosis of coronary arteries with a myocardial infarction development occurs in 5,3-12% of patients. In this clinical case patients have to be treated according to the guidelines of management of acute coronary syndrome, however they also have to start pathogenetic treatment. The article provides a clinical example illustrating the need for early pre-asymptomatic diagnosis of ET. In this clinical case, modern algorithms for the diagnosis and treatment of such complications of ET as myocardial infarction are considered.
Conclusions. From the above we can conclude that early asymptomatic diagnosis of ET is needed for stratification of the risk of thromboembolic complications according to new recommendations with timely startetd pathogenetic therapy.
2. Ayalew Tefferi, Alessandro M. Vannucchi & Tiziano Barbui. Essential thrombocythemia treatment algorithm 2018 // Blood Cancer Journal. 2018; 8 (1): Article number 2. doi: 10.1038/s41408-017-0041-8
3. Lyubarets TF, Prokopenko IN, Klimenko SV, Mishchenyuk OY. [Modern aspects of pathogenesis and diagnosis of Ph’-negative chronic myeloproliferative diseases] // Oncohematology. 2012; 7 (3). [in Ukrainian]. Access mode. https://www.clinicaloncology.com.ua/article/4641/suchasni-aspekti-patogenezu-ta-diagnostiki-ph-negativnix-xronichnix-miyeloproliferativnix-zaxvoryuvan
4. Eftychia Demerouti, Athanassios Manginas, Evangelos Leontiadis et al. A 53-year-old woman with thrombocytosis and pulmonary embolism // Arch Med Sci. 2014; 10(1): 191-4. https:// doi: 10.5114/aoms.2014.40745
5. Barbui T., Thiele J., Gisslinger H. et al. The 2016 revision of WHO classification of myeloproliferative neoplasms: clinical and molecular advances // Blood Reviews. 2016; 30 (6): 453-9. https://doi.org/10.1016/j.blre.2016.06.001
6. Pósfai É, Marton I, Szőke A et al. Stroke in essential thrombocythemia. J Neurol Sci. 2014;336:260–2.
7. Michiels JJ, Dulicek P, Berneman Z, Gadisseur A, Schroyens W. Venous Thrombophilia, Platelet von Willebrand Factor Mediated Arteriolar Microvascular Thrombosis in JAK2V617F Mutated Thrombocythemia and Acquired ADAMTS13 Deficiency as Causes of Intrahepatic Obstructive Microvascular Liver Diseases in Budd-Chiari Syndrome and Splanchnic Vein Thrombosis // J Cardiol and Cardiovasc Sciences. 2018; 2(5): 33-45. DOI: 10.29245/2578-3025/2018/5.1154
8. Michiels J.J., Berneman Z., Schroyens W., Finazzi G., Budde U., van Vliet H.H. The Paradox of Platelet Activation and Impaired Function: Platelet-Von Willebrand Factor Interactions, and the Etiology of Thrombotic and Hemorrhagic Manifestations in Essential Thrombocythemia and Polycythemia Vera // Semin Thromb Hemost. 2006; 32(6): 589-604. doi: 10.1055/s-2006-949664.
9. Melillo L, Tieghi A, Candoni A, Radaelli F, Ciancia R, et al. Outcome of 122 pregnancies in essential thrombocythemia patients: A report from the Italian registry // Am J Hematol. 2009; 84(10): 636-40. doi: 10.1002/ajh.21504.
10. Pósfai É, Marton I, Borbényi Z, Nemes A. Myocardial infarction as a thrombotic complication of essential thrombocythemia and polycythemia vera // Anatol J Cardiol. 2016; 16(6): 397-402. https:// doi: 10.14744/AnatolJCardiol.2015.6125
11. Camachoa FJ, Hernándeza N, Díaza E. et al. Essential thrombocythemia and acute myocardial infarction // Advanced Search. 2009; 62 (5): 583-5. DOI: 10.1016/S1885-5857(09)71846-8
12. Soucy-Giguère MC, Turgeon PY, Sénéchal M. What cardiologists should know about essential thrombocythemia and acute myocardial infarction: report of two cases and advanced heart failure therapies considerations // International Medical Case Reports Journal. 2019; 12: 253-9. https://doi.org/10.2147/IMCRJ.S217568
13. Purva Sharma, Sameer Gupta, Pankit Patel, et al. Acute ST-segment elevation myocardial infarction as the first manifestation of essential thrombocytosis // Cureus Journal of Medical Science. 2019; 11(2): e4032. doi:10.7759/cureus.4032
14. Kristinsson S.Y., Landgren O. et al. Autoimmunity and the risk of myeloproliferative neoplasms // Haematologica. 2010; 95(7): 1216-20. https://doi: 10.3324/haematol.2009.020412
15. Barbui T., Finazzi G., Carobbio A., et al. Development and validation of an international prognostic score of thrombosis in World Health Organization-essential thrombocythemia (IPSET-thrombosis) // Blood. 2012; 120(26): 5128-33. doi: 10.1182/blood-2012-07-444067.
16. Palandri F, Polverelli N, Catani L, Sollazzo D, et al. Bleeding in essential thrombocythaemia: a retrospective analysis on 565 patients // British journal of heamatology. 2011; 156(2): 281-4. https://doi.org/10.1111/j.1365-2141.2011.08858.x
17. Kleman A, Singavi AK, Michaelis LC. Current Challenges in the Management of Essential Thrombocythemia // Clinical Advances in Hematology & Oncology. 2017; 15 (10): 773-83.
This work is licensed under a Creative Commons Attribution 4.0 International License.