Клинические особенности и диагностика Ph-негативных миелопролиферативных заболеваний, протекающих совместно с антифосфолипидным синдромом
Клинические особенности и диагностика Ph-негативных миелопролиферативных заболеваний, протекающих совместно с антифосфолипидным синдромом
Меликян А.Л., Суборцева И.Н, Колошейнова Т.И. и др. Клинические особенности и диагностика Ph-негативных миелопролиферативных заболеваний, протекающих совместно с антифосфолипидным синдромом. Терапевтический архив. 2019; 91 (7): 93–99. DOI: 10.26442/00403660.2019.07.000324
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Melikyan A.L., Subortseva I.N., Koloshejnova E.A., et al. Clinical features and diagnosis of Ph-negative myeloproliferative neoplasms occurring in conjunction with the antiphospholipid syndrome. Therapeutic Archive. 2019; 91 (7): 93–99. DOI: 10.26442/00403660.2019.07.000324
Клинические особенности и диагностика Ph-негативных миелопролиферативных заболеваний, протекающих совместно с антифосфолипидным синдромом
Меликян А.Л., Суборцева И.Н, Колошейнова Т.И. и др. Клинические особенности и диагностика Ph-негативных миелопролиферативных заболеваний, протекающих совместно с антифосфолипидным синдромом. Терапевтический архив. 2019; 91 (7): 93–99. DOI: 10.26442/00403660.2019.07.000324
________________________________________________
Melikyan A.L., Subortseva I.N., Koloshejnova E.A., et al. Clinical features and diagnosis of Ph-negative myeloproliferative neoplasms occurring in conjunction with the antiphospholipid syndrome. Therapeutic Archive. 2019; 91 (7): 93–99. DOI: 10.26442/00403660.2019.07.000324
Тромбоз является тяжелым и крайне опасным заболеванием, которое оказывает негативное влияние на качество и продолжительность жизни. Антифосфолипидный синдром (АФС) характеризуется повторяющимися тромбозами венозного, артериального, микроциркуляторного русла, патологией беременности с потерей плода и синтезом антифосфолипидных антител. Высокий риск тромботических осложнений также наблюдается у больных миелопролиферативными заболеваниями (МПЗ). Представлено описание трех клинических случаев Ph-негативных МПЗ, протекающих совместно с АФС. Во всех случаях рецидивирующие тромбозы позволили заподозрить наличие двух заболеваний – МПЗ и АФС.
Thrombosis is a serious and extremely dangerous disease that has a negative impact on the quality and longevity. Antiphospholipid syndrome (APS) is a pathology characterized by recurring venous, arterial, microvasculature thrombosis, pregnancy pathology with loss of the fetus and the synthesis of antiphospholipid antibodies. A high risk of thrombotic complications is also observed in patients with myeloproliferative neoplasms (MPN). This article presents a description of three clinical cases of Ph-negative myeloproliferative diseases, occurring in conjunction with APS. In all cases, recurrent thrombosis allowed to suspect the presence of two diseases – MPN and APS.
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1. [Melikyan AL, Turkina AG, Abdulkadyrov KM, Zaritsky AYu, Afanasyev BV, Shuvaev VA, Lomaia EG, Morozova EV, Baikov VV, Golenkov AK, Subortseva IN, Sokolova MA, Kovrigina AM, Martynkevich IS, Gritsaev SV, Sudarikov AB, Sukhanova GA, Ivanova VL, Kaplanov KD, Konstantinova TS, Pospelova TI, Ageeva TA, Shatokhin YuV, Savchenko VG. Clinical recommendations for the diagnosis and therapy of Ph-negative myeloproliferative diseases (true polycythemia, essential thrombocythemia, primary myelofibrosis). Gematologia and transfuziologiya. 2014;59(4):31-56 (In Russ.)].
2. [Melikyan AL, Turkina AG, Kovrigina AM, Subortseva IN, Sudarikov AB, Sokolova MA, Zaritskiy AYu, Lomaia EG, Shuvaev VA, Gritsaev SV, Martynkevich IS, Afanasiev BV, Morozova EV, Baykov VV, Golenkov AK, Ivanova VL, Kaplanov KD, Pospelova TI, Ageeva TA, Shatokhin YuV, Savchenko VG. Clinical recommendations for the diagnosis and therapy of Ph-negative myeloproliferative neoplasms (polycythemia vera, essential thrombocythemia, primary myelofibrosis) (edition 2016). Gematologia and transfuziologiya. 2017;62(1):25-60 (In Russ.)].
3. Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A, Harris NL, Le Beau MM, Hellström-Lindberg E, Tefferi A, Bloomfield CD. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood. 2009;114(5):937-51. doi:10.1182/blood-2009-03-209262
4. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, Bloomfield CD, Cazzola M, Vardiman JW. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127(20):2391-405. doi: 10.1182/blood-2016-03-643544
5. Crisa E, Venturino E, Passera R, Prina M, Schinco P, Borchiellini A, Giai V, Ciocca Vasino MA, Bazzan M, Vaccarino A, Boccadoro M, Ferrero DA. Retrospective study on 226 polycythemia vera patients: impact of median hematocrit value on clinical outcomes and survival improvement with anti-thrombotic prophylaxis and non-alkylating drugs. Ann Hematol. 2010;89(7):691-99. doi: 10.1007/s00277-009-0899-z
6. [Subortseva IN, Kolosheinova TI, Pustovaya EI, Egorova EK, Kovrigina AM, Pliskunova YuV, Makarik TV, Abdullaev AO, Melikyan AL. Polycythemia Vera: Literature Review and Own Data. Klinicheskaya Onkogematologiya. Fundamental`nye issledovaniya I klinicheskaya ptaktika. 2015;8(4):397-412 (In Russ.)].
7. Tfferi A, Rumi E, Finazzi G, Gisslinger H, Vannucchi AM, Rodeghiero F, Randi ML, Vaidya R, Cazzola M, Rambaldi A, Gisslinger B, Pieri L, Ruggeri M, Bertozzi I, Sulai NH, Casetti I, Carobbio A, Jeryczynski G, Larson DR, Müllauer L, Pardanani A, Thiele J, Passamonti F, Barbui T. Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. Leukemia. 2013;27(9):1874-81. doi: 10.1038/leu.2013.163
8. Barbui T, Finazzi G, Carobbio A, Thiele J, Passamonti F, Rumi E. Rodeghiero F, Randi ML, Bertozzi I, Gisslinger H, Buxhofer-Ausch V, De Stefano V, Betti S, Rambaldi A, Vannucchi AM, Tefferi A. 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
9. Passamonti F, Rumi E, Pungolino E, Malabarba L, Bertazzoni P, Valentini M, Orlandi E, Arcaini L, Brusamolino E, Pascutto C, Cazzola M, Morra E, Lazzarino M. Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia. Am J Med. 2004;117(10):755-61. doi: 10.1016/j.amjmed.2004.06.032
10. Carobbio A, Finazzi G, Guerini V, Spinelli O, Delaini F, Marchioli R, Borrelli G, Rambaldi A, Barbui T. Leukocytosis is a risk factor for thrombosis in essential thrombocythemia: interaction with treatment, standard risk factors and Jak2 mutation status. Blood. 2007;109(6):2310-3. doi:10.1182/blood-2006-09-046342
11. Wolanskyj AP, Schwager SM, McClure RF, Larson DR, Tefferi A. Essential thrombocythemia beyond the first decade: life expectancy, long-term complication rates, and prognostic factors. Mayo Clin Proc. 2006; 1(2):159-66. doi:10.4065/81.2.159
12. Vannucchi AM, Antonioli E, Guglielmelli P, Rambaldi A, Barosi G, Marchioli R, Marfisi RM, Finazzi G, Guerini V, Fabris F, Randi ML, De Stefano V, Caberlon S, Tafuri A, Ruggeri M, Specchia G, Liso V, Rossi E, Pogliani E, Gugliotta L, Bosi A, Barbui T. Clinical profile of homozygous JAK2 617V>F mutation in patients with polycythemia vera or essential thrombocythemia. Blood. 2007;110(3):840-6. doi:10.1182/blood-2006-12-064287
13. Alvarez-Larrán A, Cervantes F, Bellosillo B, Giralt M, Juliá A, Hernández-Boluda JC, Bosch A, Hernández-Nieto L, Clapés V, Burgaleta C, Salvador C, Arellano-Rodrigo E, Colomer D, Besses C. Essential thrombocythemia in young individuals: frequency and risk factors for vascular events and evolution to myelofibrosis in 126 patients. Leukemia. 2007;21(6):1218-23. doi:10.1038/sj.leu.2404693
14. Wolanskyj AP, Schwager SM, McClure RF, Larson DR,Tefferi A. .Essential thrombocythemia beyond the first decade: life expectancy, long-term complication rates, and prognostic factors. Mayo Clin Proc. 2006;81(2):159-66. doi: 10.4065/81.2.159
15. [Melikyan AL, Subortseva IN. Biology of myeloproliferative malignancies. Klinicheskaya onkogematologiya. Fundamental`nye issledovaniya I klinicheskaya ptaktika. 2016;9(3):314-25 (In Russ.)]. doi: 10.1182/blood-2009-03-209262
16. [Shikhbabaeva DI, Polushkina LB, Shuvaev VA, Martynkevich IS, Kapustin SI, Zamotina TB, Fominykh MS, Udal’eva VU, Zotova II, Shmeleva VM, Smirnova OA, Voloshin SV, Bessmel’tsev SS, Chechetkin AV, Abdulkadyrov KM. Genetic markers of hereditary thrombophilia and risk of thrombotic complications in patients with polycythemia vera. Klinicheskaya onkogematologiya. Myeloid tumors. 2017;10(1):85-92 (In Russ.)]. doi: 10.21320/2500-2139-2017-10-1-85-92
17. [Reshetnyak TM. Antiphospholipid syndrome: diagnosis and clinical manifestations (a lecture). Nauchno-prakticheskaya revmatologiya. 2014;5(1):56-71 (In Russ.)]. doi:10.14412/ 1995-4484-2014-56-71
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19. Miyakis S, Lockshin M, Atsumi T, Branch DW, Brey RL, Cervera R, Derksen RH, DE Groot PG, Koike T, Meroni PL, Reber G, Shoenfeld Y, Tincani A, Vlachoyiannopoulos PG, Krilis SA. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4(2):295-306. doi:10.1111/j.1538-7836.2006.01753.x
20. Anakwue RC, Chijioke C, Mbah A, Onuh A, Okwara C. Antiphospholipid syndrome in a young Nigerian girl presenting with gangrenous toes. Cardiovasc J Afr. 2013;24(9-10):8-11. doi: 10.5830/ CVJA-2013-073
21. Montoya RC, Gajra A. Current status of new anticoagulants in the management of venous thromboembolism. Advances in Hematol. 2012; 2012(856341):7 pages. doi:10,1155/2012/856341
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