Tuğba ÇETİNTEPE, Lütfi ÇETİNTEPE, Cansu Atmaca MUTLU, Şerife SOLMAZ, CELAL ACAR, Sinem NAMDAROĞLU, Füsun GEDİZ

ESANSİYEL TROMBOSİTOZ TANILI HASTALARIMIZIN RETROSPEKTİF DEĞERLENDİRİLMESİ

Giriş: Çalışmamızda Sağlık Bilimleri Üniversitesi Bozyaka Eğitim ve Araştırma Hastanesi Hematoloji Kliniğinde takip edilen 118 Esansiyel trombositozlu (ET) hastanın demografik verileri, eşlik eden mutasyon durumları, kullandığı tedaviler ve sağkalım analizlerinin yapılması amaçlandı. Gereç ve Yöntem: Esansiyel Trombositoz tanısı ile izlenen 118 olgunun verileri geriye dönük olarak değerlendirildi. Bulgular: Hastaların 68’i kadın (% 58), 50‘si erkek (%42 )idi. Hastaların ortanca yaş değeri 68 yaş idi. 118 hastanın 59 ‘unda JAK2 mutasyonu pozitif bulundu. Altı yıllık izlemde 22 hastada (%18) trombotik olay izlendi. JAK2 mutasyonu pozitif olan hastaların %13’ünde trombotik olay gözlendi. Serebrovasküler olay en çok görülen trombotik olaydı. 22 hastanın 13’ünde (%59) serebrovasküler olay saptandı. JAK2 mutasyonu pozitif olan hastalarda trombotik olayların daha sık olması istatiksel olarak anlamlı bulundu (p=0,004). Sonuç: Hastalarımızın demografik özellikleri diğer çalışmalar ile benzerdi. JAK2 mutasyonu pozitif olan hastalarda trombotik olaylar daha sık gözlenmektedir. Çalışmamızda JAK2 mutasyonu ile tromboz arasında anlamlı bir ilişki bulunmuştur.

RETROSPECTIVE EVALUATION OF OUR PATIENTS WITH DIAGNOSIS ESSENTIAL THROMBOCYTHEMIA

Introduction: The aim of this study was to evaluate the demographic data, accompanying mutations, treatments and survival analyzes of 118 patients with essential thrombocytosis who were followed up in our department in the Hematology Clinic of Bozyaka Training and Research Hospital. Material and Method: Data of 118 ET patients were evaluated retrospectively. Results: We found that 68 of the patients were woman and 50 of the patients were man. And median age were 68. Of the 118 patients, 59 had positive JAK2 mutations. Thrombotic events were observed in 22 patients (18%) at the 6-year follow-up. Thrombotic events were observed in 13% of JAK2 mutation-positive patients. Cerebrovascular events were the most common thrombotic events. Cerebrovascular events were detected in 13 (59%) of the 22 patients. It was statistically significant that thrombotic events were more frequent in JAK2 mutation-positive patients (p = 0.004). Conclusion: The demographic characteristics of our patients were similar to those of other studies. Thrombotic events are more common in patients with positive JAK2 mutations. There was a significant correlation between JAK2 mutation and thrombosis in our study

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Mesa RA, Silverstein MN, Jacobsen SJ, Wollan PC, Tefferi A. Population-based incidence and survival figures in essential thrombocythemia and agnogenic myeloid metaplasia: an Olmsted County Study, 1976-1995. Am J Hematol 1999; 61(1): 10-5.
Barbui T, Thiele J, Gisslinger H, Finazzi G, Vannucchi AM, Tefferi A. The 2016 revision of WHO classification of myeloproliferative neoplasms: Clinical and molecular advances. Blood Rev 2016; 30(6): 453-9.
Tefferi A. Novel mutations and their functional and clinical relevance in myeloproliferative neoplasms: JAK2, MPL, TET2, ASXL1, CBL, IDH and IKZF1. Leukemia 2010; 24(6): 1128-38.
Alvarez-Larrán A, Cervantes F, Pereira A, Arellano-Rodrigo E, Pérez-Andreu V, Hernández-Boluda JC, et al. Observation versus antiplatelet therapy as primary prophylaxis for thrombosis in low-risk essential thrombocythemia. Blood 2010; 116(8): 1205-10.
Bayram M, Özkocaman V, Özkalemkaş F. Uludağ Üniversitesi Tıp Fakültesi Dergisi 2011; 13(3): 16-5.
Anand S, Stedham F, Beer P, Gudgin E, Ortmann CA, Bench A, et al. Effects of the JAK2 mutation on the hematopoietic stem and progenitor compartment in human myeloproliferative neoplasms. Blood. 2011; 118(1): 177-81.
Wolanskyj AP, Lasho TL, Schwager SM, McClure RF, Wadleigh M, Lee SJ, et al. JAK2 mutation in essential thrombocythaemia: clinical associations and long-term prognostic relevance. Br J Haematol 2005; 131(2): 208-13.
Tefferi A. Classification, diagnosis and management of myeloproliferative disorders in the JAK2V617F era. Hematology Am Soc Hematol Educ Program 2006; 240-5.
Finazzi G, Rambaldi A, Guerini V, Carobbo A, Barbui T. Risk of thrombosis in patients with essential thrombocythemia and polycythemia vera according to JAK2 V617F mutation status. Haematologica 2007;92(1): 135-6.
Vanucchi AM, Antonioli E, Guglielmelli P. Clinical profile of homozygous JAK 2 V617F mutation in patients with polcythemia vera or essential thrombocythemia. Blood 2007; 110(3): 840-6.
Lussana F, Caberlon S, Pagani C, Kamphuisen PW, Büller HR, Cattaneo M. Association of V617F Jak2 mutation with the risk of thrombosis among patients with essential thrombocythaemia or idiopathic myelofibrosis: a systematic review. Thromb Res 2009; 124(4): 409-17.
Campbell PJ, Green AR. The myeloproliferative disorders. N Engl J Med 2006; 355(23): 2452-66.