POLYCYTHEMIA VERA: UPDATE ON PATHOGENESIS, DIAGNOSIS AND MANAGEMENT

Year 2015, , 83 - 91, 21.03.2015

İpek Yönal , Fatma Sargın

https://doi.org/10.18017/iuitfd.13056441.2015.78/3.83-91

Abstract

Polycythemia vera (PV) is a myeloproliferative neoplasm characterized by increased red blood cell mass and usually overproduction of granulocytes and platelets and increased spleen size. JAK2V617F mutation is present in more than 95% of PV patients. Bone marrow examination reveals excessive proliferation of erythroid, myeloid, and megakaryocytic elements. The prognosis of PV depends on the severity of the complications occurring during the clinical course. Thrombotic complications are the main cause of morbidity and mortality in PV. Survival is affected whether appropriate therapy is applied during the erythrocytotic phase of the disease. Uncontrolled erythrocytosis poses very high risk for development of thrombosis. Some studies suggest that PV patients have a normal or near-normal lifeexpectancy. Most studies, however, report excess mortality caused by thrombotic complications and acute leukemia transformation during course of PV. This review aims to highlight the pathogenesis, diagnosis and current management in PV

Keywords

Acute leukemia transformation, Myeloproliferative neoplasm, Polycythemia vera, Thrombotic complications

POLİSİTEMİA VERA: PATOGENEZ, TEŞHİS VE TEDAVİDE GÜNCEL BİLGİLER

Abstract

Polisitemia vera (PV), eritrosit kitlesinde mutlak artış ile karakterize, sıklıkla granülosit ve trombositlerin aşırı üretiminin ve splenomegalinin eşlik ettiği bir miyeloproliferatif neoplazidir. JAK2V617F mutasyonu, PV tanılı olguların %95’inden fazlasında görülmektedir. Kemik iliği incelemesinde eritroid, miyeloid ve megakaryositik seride proliferasyon görülür. PV’nın prognozu, klinik seyir sırasında ortaya çıkan komplikasyonların ciddiyetine bağlıdır. PV’da morbidite ve mortalitenin ana nedeni trombotik komplikasyonlardır. Yaşam, hastalığın pletorik fazında uygun tedavinin başlanıp başlanmamasından etkilenmektedir. Kontrol edilemeyen eritrositoz varlığında tromboz riski belirgin şekilde artış gösterir. Bazı çalışmalarda PV’nın normal veya normale yakın yaşam süresi ile ilişkisi gösterilmiştir. Fakat, birçok çalışmada PV’da trombotik komplikasyonlar ve akut lösemiye dönüşüm sonucunda artmış mortalite olduğu bildirilmiştir. Bu derlemenin amacı, PV tanılı olgularda patogenez, tanı yöntemleri ve güncel tedaviyi vurgulamaktır.

Keywords

Akut lösemiye dönüşüm, Miyeloproliferatif neoplazi, Polisitemia vera, Trombotik komplikasyonlar

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