Тромбозы у больных с миелопролиферативными новообразованиями. Описание клинического случая. Клиническое наблюдение

Меликян А.Л., Суборцева И.Н., Гилязитдинова Е.А., Колошейнова Т.И., Шашкина К.С., Егорова Е.К., Ковригина А.М., Судариков А.Б., Горгидзе Л.А. Тромбозы у больных с миелопролиферативными новообразованиями. Клиническое наблюдение. Терапевтический архив. 2021; 93 (7): 800–804.
DOI: 10.26442/00403660.2021.07.200925

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Melikyan AL, Subortseva IN, Gilyazitdinova EA, Koloshejnova TI, Shashkina KS, Egorova ЕК, Kovrigina AM, Sudarikov AB, Gorgidze LA. Thrombosis in patients with myeloproliferative neoplasms. Case report. Terapevticheskii Arkhiv (Ter. Arkh.). 2021; 93 (7): 800–804. DOI: 10.26442/00403660.2021.07.200925

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Аннотация

Тромботические осложнения – значимые факторы, определяющие прогноз при миелопролиферативных новообразованиях. Маркерами, позволяющими оценить риск развития тромбозов, являются количество лейкоцитов, тромбоцитов, уровень гемоглобина, гематокрит, возраст, молекулярный статус, тромбозы в анамнезе, ожирение, артериальная гипертензия, гиперлипидемия, наследственная или приобретенная тромбофилия. Патогенез тромбозов у больных с миелопролиферативными новообразованиями сложный и многофакторный. В большинстве случаев этиологический фактор остается неизвестным. В настоящее время терапия антиагрегантами и антикоагулянтами проводится в индивидуальном порядке. Алгоритм первичной и вторичной (после состоявшегося тромбоза) профилактики требует разработки и апробации. Мы представляем клиническое наблюдение – повторные артериальные и венозные тромботические осложнения у больного с первичным миелофиброзом.

Ключевые слова: миелопролиферативное новообразование, тромбозы, первичный миелофиброз

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Thrombotic complications are the most significant factors determining the prognosis in myeloproliferative neoplasms. Markers for assessing the risk of thrombosis are the number of leukocytes, platelets, hemoglobin level, hematocrit, age, molecular status, history of thrombosis, obesity, arterial hypertension, hyperlipidemia, hereditary or acquired thrombophilia. The pathogenesis of thrombosis in patients with myeloproliferative neoplasms is complex and multifactorial. In most cases, the etiological factor remains unknown. Currently, antiplatelet and anticoagulant therapy is carried out on an individual basis. The algorithm for primary and secondary (after thrombosis) prevention requires development and testing. We present a clinical case of repeated arterial and venous thrombotic complications in a patient with primary myelofibrosis.

Keywords: myeloproliferative neoplasms, thrombosis, primary myelofibrosis

Список литературы

1. Меликян А.Л., Ковригина А.М., Суборцева И.Н., и др. Национальные клинические рекомендации по диагностике и терапии Ph-негативных миелопролиферативных заболеваний (истинная полицитемия, эссенциальная тромбоцитемия, первичный миелофиброз). Гематология и трансфузиология. 2018;63(3):275-315 [Melikyan AL, Kovrigina AM, Subortseva IN, et al. National сlinical recommendations for diagnosis and therapy of Ph-negative myeloproliferative neoplasms (polycythemia vera, essential thrombocythemia, primary myelofibrosis). Hematology and Transfusiology. 2018;63(3):275-315 (in Russian)]. DOI:10.25837/HAT.2019.51.88.001
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5. Sukhanova GA, Melikyan AL, Vakhrusheva MV, et al. Treatment of portal thrombosis in patients with myeloproliferative neoplasms: a single-institution experience. Blood. 2014;124(21):5098. DOI:10.1182/blood.V124.21.5098.5098
6. Rungjirajittranon T, Owattanapanich W, Ungprasert P, Ruchutrakool T. A systematic review and meta-analysis of the prevalence of thrombosis and bleeding at diagnosis of Philadelphia-negative myeloproliferative neoplasms. BMC Cancer. 2019;19(1):184. DOI:10.1186/s12885-019-5387-9
7. Marchioli R, Finazzi G, Landolfi R, et al. Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. J Clin Oncol. 2005;23(10):2224-32. DOI:10.1200/JCO.2005.07.062
8. 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):5128-33. DOI:10.1182/blood-2012-07-444067
9. Cervantes F, Arellano-Rodrigo E, Alvarez-Larrán A. Blood cell activation in myeloproli-ferative neoplasms. Haematologica. 2009:94(11):1484-88. DOI:10.3324/haematol.2009.013375
10. Chung I, Lip GY. Virchow's triad revisited: blood constituents. Pathophysiol Haemost Thromb. 2003;33(5-6):449-54. DOI:10.1159/000083844
11. Michiels JJ, Berneman Z, Schroyens W, et al. 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):589-604. DOI:10.1055/s-2006-949664
12. Меликян А.Л., Суборцева И.Н. Протокол дифференцированной терапии молодых больных истинной полицитемией и эссенциальной тромбоцитемией. В кн.: Абрамова А.В., Абдуллаев А.О., Азимова М.Х., и др. Алгоритмы диагностики и протоколы лечения заболеваний системы крови. В 2 т. М., 2018; c. 825-44 [Melikyan AL, Subortseva IN. Protocol of differentiated therapy of young patients with polycythemia vera and essential thrombocytemia. In: Abramova AV, Abdullaev AO, Azimova MKh, et al. Diagnostic algorithms and protocols for the treatment of the blood system diseases. Moscow, 2018; p. 825-44 (in Russian)].
13. Finazzi G. A prospective analysis of thrombotic events in the European collaboration study on low-dose aspirin in polycythemia (ECLAP). Pathol Biol (Paris). 2004;52(5):285-8. DOI:10.1016/j.patbio.2004.02.007
14. Marchioli R, Finazzi G, Specchia G, et al. Cardiovascular Events and Intensity of Treatment in Polycythemia Vera. N Engl J Med. 2013;(368):22‑33. DOI:10.1056/NEJMoa1208500
15. Alvarez-Larrán A, Pereira A, Guglielmelli P, et al. Antiplatelet therapy versus observation in low-risk essential thrombocythemia with a CALR mutation. Haematologica. 2016;101(8):926-31. DOI:10.3324/haematol.2016.146654
16. Verstovsek S, Vannucchi AM, Griesshammer M, et al. Ruxolitinib versus best available therapy in patients with polycythemia vera: 80 week follow up from the RESPONSE trial. Haematologica. 2016;101(7):821-29. DOI:10.3324/haematol.2016.143644
17. Samuelson BT, Vesely SK, Chai-Adisaksopha C, et al. The impact of ruxolitinib on thrombosis in patients with polycythemia vera and myelofibrosis: a meta-analysis. Blood Coagul Fibrinolysis. 2016;27(6):648‑52. DOI:10.1097/MBC.0000000000000446
18. De Stefano V, Ruggeri M, Cervantes F, et al. High rate of recurrent venous thromboembolism in patients with myeloproliferative neoplasms and effect of prophylaxis with vitamin K antagonists. Leukemia. 2016;30(10):2032-8. DOI:10.1038/leu.2016.85
19. Arachchillage DR, Laffan M. Pathogenesis and Management of Thrombotic Disease in Myeloproliferative Neoplasms. Semin Thromb Hemost. 2019;45(6):604-11. DOI:10.1055/s-0039-1693477
20. Serrao A, Breccia M, Napolitano M, et al. A multicenter real life study on anticoagulant treatment with direct oral anticoagulants in patients with Ph-negative myeloproliferative neoplasms. AJH. 2020; 9(12):329-32. DOI:10.1002/ajh.25990

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1. Melikyan AL, Kovrigina AM, Subortseva IN, et al. National сlinical recommendations for diagnosis and therapy of Ph-negative myeloproliferative neoplasms (polycythemia vera, essential thrombocythemia, primary myelofibrosis). Hematology and Transfusiology. 2018;63(3):275-315 (in Russian) DOI:10.25837/HAT.2019.51.88.001
2. Melikyan AL, Subortseva IN, Baryshnikova DV, et al. Thrombotic and hemorrhagic complications in patients with primary myelofibrosis. Data from the register of Ph-negative myeloproliferative diseases. Hematology and Transfusiology. 2020;6 5(S1):38 (in Russian)
3. Kc D, Falchi L, Verstovsek S. The underappreciated risk of thrombosis and bleeding in patients with myelofibrosis: a review. Ann Hematol. 2017;96(10):1595-604. DOI:10.1007/s00277-017-3099-2
4. Tanashyan MM, Kuznetsova PI, Subortseva IN, et al. Chronic and acute cerebrovascular pathology in patients with ph-negative myeloproliferative diseases. Hematology and Transfusiology. 2016;61(3):146‑50 (in Russian) DOI:10.18821/0234-5730-2016-61-3-146‑150
5. Sukhanova GA, Melikyan AL, Vakhrusheva MV, et al. Treatment of portal thrombosis in patients with myeloproliferative neoplasms: a single-institution experience. Blood. 2014;124(21):5098. DOI:10.1182/blood.V124.21.5098.5098
6. Rungjirajittranon T, Owattanapanich W, Ungprasert P, Ruchutrakool T. A systematic review and meta-analysis of the prevalence of thrombosis and bleeding at diagnosis of Philadelphia-negative myeloproliferative neoplasms. BMC Cancer. 2019;19(1):184. DOI:10.1186/s12885-019-5387-9
7. Marchioli R, Finazzi G, Landolfi R, et al. Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. J Clin Oncol. 2005;23(10):2224-32. DOI:10.1200/JCO.2005.07.062
8. 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):5128-33. DOI:10.1182/blood-2012-07-444067
9. Cervantes F, Arellano-Rodrigo E, Alvarez-Larrán A. Blood cell activation in myeloproli-ferative neoplasms. Haematologica. 2009:94(11):1484-88. DOI:10.3324/haematol.2009.013375
10. Chung I, Lip GY. Virchow's triad revisited: blood constituents. Pathophysiol Haemost Thromb. 2003;33(5-6):449-54. DOI:10.1159/000083844
11. Michiels JJ, Berneman Z, Schroyens W, et al. 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):589-604. DOI:10.1055/s-2006-949664
12. Melikyan AL, Subortseva IN. Protocol of differentiated therapy of young patients with polycythemia vera and essential thrombocytemia. In: Abramova AV, Abdullaev AO, Azimova MKh, et al. Diagnostic algorithms and protocols for the treatment of the blood system diseases. Moscow, 2018; p. 825-44 (in Russian)
13. Finazzi G. A prospective analysis of thrombotic events in the European collaboration study on low-dose aspirin in polycythemia (ECLAP). Pathol Biol (Paris). 2004;52(5):285-8. DOI:10.1016/j.patbio.2004.02.007
14. Marchioli R, Finazzi G, Specchia G, et al. Cardiovascular Events and Intensity of Treatment in Polycythemia Vera. N Engl J Med. 2013;(368):22‑33. DOI:10.1056/NEJMoa1208500
15. Alvarez-Larrán A, Pereira A, Guglielmelli P, et al. Antiplatelet therapy versus observation in low-risk essential thrombocythemia with a CALR mutation. Haematologica. 2016;101(8):926-31. DOI:10.3324/haematol.2016.146654
16. Verstovsek S, Vannucchi AM, Griesshammer M, et al. Ruxolitinib versus best available therapy in patients with polycythemia vera: 80 week follow up from the RESPONSE trial. Haematologica. 2016;101(7):821-29. DOI:10.3324/haematol.2016.143644
17. Samuelson BT, Vesely SK, Chai-Adisaksopha C, et al. The impact of ruxolitinib on thrombosis in patients with polycythemia vera and myelofibrosis: a meta-analysis. Blood Coagul Fibrinolysis. 2016;27(6):648‑52. DOI:10.1097/MBC.0000000000000446
18. De Stefano V, Ruggeri M, Cervantes F, et al. High rate of recurrent venous thromboembolism in patients with myeloproliferative neoplasms and effect of prophylaxis with vitamin K antagonists. Leukemia. 2016;30(10):2032-8. DOI:10.1038/leu.2016.85
19. Arachchillage DR, Laffan M. Pathogenesis and Management of Thrombotic Disease in Myeloproliferative Neoplasms. Semin Thromb Hemost. 2019;45(6):604-11. DOI:10.1055/s-0039-1693477
20. Serrao A, Breccia M, Napolitano M, et al. A multicenter real life study on anticoagulant treatment with direct oral anticoagulants in patients with Ph-negative myeloproliferative neoplasms. AJH. 2020; 9(12):329-32. DOI:10.1002/ajh.25990

Авторы

А.Л. Меликян*, И.Н. Суборцева, Е.А. Гилязитдинова, Т.И. Колошейнова, К.С. Шашкина, Е.К. Егорова, А.М. Ковригина, А.Б. Судариков, Л.А. Горгидзе

ФГБУ «Национальный медицинский исследовательский центр гематологии» Минздрава России, Москва, Россия
*anoblood@mail.ru

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Anait L. Melikyan*, Irina N. Subortseva, Elena A. Gilyazitdinova, Tamara I. Koloshejnova, Kristina S. Shashkina, Elena К. Egorova, Alla M. Kovrigina, Andrei B. Sudarikov, Lana A. Gorgidze

National Research Center for Hematology, Moscow, Russia
*anoblood@mail.ru

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