Polisitemia vera hastalığında güncel tedavi yaklaşımları
Anahtar Kelimeler:
Polisitemia Vera- JAK2 V617F- Miyeloproliferatif HastalıklarÖzet
Polisitemia vera (PV), eritrosit miktarındaki artışla tanımlanır ve sıklıkla trombosit ve granülosit üretiminin aşırı seviyede olduğu ve splenomegali ile birlikte gelen bir miyeloproliferatif hastalıktır. JAK2V617F mutasyonu, PV tanısı konan hastaların %95'inden fazlasında bulunur. Kemik iliğinde eritroid, miyeloid ve megakaryositik hücrelerde artış görülür. PV'nin sonuçları hastalık sürecinde ortaya çıkan komplikasyonların ciddiyetine bağlıdır. Trombotik komplikasyonlar, PV'de morbidite ve mortalitenin başlıca nedenidir. İyi tedavinin zamanında başlanması, hastalığın süresini etkiler. Eritrositozun kontrol edilememesi tromboz riskini artırır. PV'nin normal yaşam süresi ile ilişkisi bazı çalışmalarda gösterilmiştir, ancak trombotik komplikasyonlar ve akut lösemiye dönüşüm sonucu artan mortalite de bildirilmiştir. Bu çalışma, PV tanısı konan hastalarda patogenez, tanı yöntemleri ve güncel tedaviyi vurgulamayı amaçlamaktadır.
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