Esansiyel Trombositemi Hastalarının Geriye Dönük Değerlendirilmesi

Öz

Çalışmamızda esansiyel trombositemi (ET) tanılı hastalarda klinik, laboratuvar bulguları, tedavileri ile kanama ve tromboz durumlarını etkileyen faktörleri araştırmayı amaçladık. 2016- 2022 arası ET tanısı alan 270 hasta retrospektif incelendi, yaş ortalaması median 59 (19-92), %64’ü kadındı. Yüksek risk skoruna sahip olan JAK2 mutasyon pozitifliği %59 olup hastaların %39’ unda tromboz geliştiği (p=0.001), %23’ ünde kanama olduğu (p=0.008) saptandı. Ayrıca JAK2 pozitif olan hastaların çoğunun 60 yaş üstü olduğu (p=0.05), daha yüksek oranda miyelofibrozis geliştiği (p=0.007) görüldü. JAK2 mutasyonu pozitif hastaların, tanıda daha yüksek trombosit ve hemoglobin (Hb) değerine sahip olduğu bulundu (p=0.05, p=0.013). Hastalarımızın %33’ünde tanıda tromboz vardı (%89 arteryel ve %11 venöz trombozdu). Trombozu etkileyen risk durumları incelendiğinde; lökositoz, yüksek nötrofil ve Hb değeri ile istatistiksel anlamlılık saptandı (p=0.037, p=0.030, p=0.039). Multivariete analiz yapıldığında ise tromboz ile jak-2 pozitifliği ve lökositozun olması anlamlılığını koruyordu (p=0.022, p=0.006). Hastalarımızdan 217’si sağdı (%80). 10 yıllık sağkalım %75 olup, 60 yaş üstü hastalarda (p=0.001) ve tromboz olan hasta grubunda toplam sağkalım (OS) daha kısaydı (p=0.026). ELN risk skoru yüksek bulunan hastalarda sağkalım istatistiksel olarak anlamlı şekilde düşük saptandı (p=0.001) Revise-İPSET’ e göre orta ve yüksek skora sahip olanlarda sağkalım istatistiksel olarak anlamlı şekilde daha kısa bulundu (p=0.006). ET senelerce asemptomatik seyir gösterebileceği gibi, hayatı tehdit edebilen tromboembolik komplikasyonlar ve hemorajik durumlar ile karşımıza çıkmaktadır. Bu nedenle ET hastalarının yönetimi ve tedavi ihtiyacı olan yüksek riske sahip grubu tespit etmek önem taşımaktadır.

Anahtar Kelimeler

• Esansiyel Trombositemi
• Kanama
• Tromboz
• Sağkalım

Retrospective Evaluation of Essential Thrombocythemia Patients

Öz

In our study, we aimed to investigate the clinical and laboratory findings, treatments, and factors affecting bleeding and thrombosis in patients with essential thrombocythemia (ET). A retrospective analysis was performed on 270 patients diagnosed with ET between 2016 and 2022. The median mean age was 59 years (19-92 years), and 64% were female. High-risk JAK2 mutation positivity was found in 59% of patients, and thrombosis developed in 39% of patients (p=0.001), and bleeding occurred in 23% (p=0.008). Furthermore, most JAK2-positive patients were over 60 years of age (p=0.05), and they developed myelofibrosis at a higher rate (p=0.007). JAK2 mutation-positive patients were found to have higher platelet and hemoglobin (Hb) values at diagnosis (p=0.05, p=0.013). Thirty-three percent of our patients had thrombosis at diagnosis (89% were arterial and 11% were venous). When risk factors affecting thrombosis were examined, statistical significance was found with leukocytosis, high neutrophil and Hb values (p=0.037, p=0.030, p=0.039). When multivariate analysis was performed, it was seen that the development of thrombosis and JAK2 positivity and leukocytosis were significantly correlated (p=0.022, p=0.006). 217 of our patients survived (80%). 10-year survival was 75%, and overall survival (OS) was shorter in patients over 60 years of age (p=0.001) and in the group with thrombosis (p=0.026). Survival was found to be statistically significantly shorter in patients with a high ELN risk score (p=0.001). According to Revise-IPSET, survival was found to be statistically significantly shorter in those with moderate and high scores (p=0.006). ET can present with asymptomatic symptoms for years or can present with life-threatening thromboembolic complications and hemorrhagic conditions. Therefore, it is important to manage ET patients and identify high-risk groups requiring treatment.

Anahtar Kelimeler

• Essential Thrombocytemia
• Bleeding
• Thrombosis
• ELN
• IPSET
• Revise-IPSET

Kaynakça

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