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Başlık: Polycythemia vera: Diagnosis, clinical course, and current management
Yazarlar: Büyükaşık, Yahya
Ar, Cem
Turgut, Mehmet
Yavuz, Selim
Saydam, Güray
Uludağ Üniversitesi/Tıp Fakültesi/İç Hastalıkları Anabilim Dalı/Hematoloji Anabilim Dalı.
Ali, Rıdvan
7201813027
Anahtar kelimeler: General & internal medicine
Polycythemia vera
Diagnosis
Prognosis
Treatment
Quality-of-life
Negative myeloproliferative neoplasms
Recombinant interferon-alpha
Essential thrombocythemia myelofibrosis
International working group
Hemostasis research gth
Tyrosine kinase jak2
Prognostic model
Predict survival
Risk-factors
Yayın Tarihi: 9-Ağu-2018
Yayıncı: TÜBİTAK
Atıf: Büyükaşık, Y. vd. (2018). ''Polycythemia vera: Diagnosis, clinical course, and current management''. Turkish Journal of Medical Sciences, 48(4), 698-710.
Özet: Very important developments related to polycythemia vera (PV) have occurred during the last two decades. The discovery of Janus kinase (JAK) 2 mutations has changed both the diagnosis and clinical management of PV. Currently JAK2 molecular testing is essential in the diagnostic work-up and JAK2 mutation positivity is a major diagnostic criterion. The discovery of JAK2 mutations suggested that abnormal JAK-STAT signaling was a pivotal feature in the pathogenesis of Philadelphia-negative myeloproliferative neoplasms. This idea led to the development of JAK inhibitors. Currently ruxolitinib, a JAK1/JAK2 inhibitor, is also approved for PV patients with hydroxyurea resistance or intolerance. International collaborations have made it possible to describe disease characteristics and evolution better. Presently it is possible to quantify the symptomatic burden of the disease and to estimate prognosis. In spite of these developments, management of PV still largely depends on estimation of thromboembolic risk and trying to decrease the risk with or without cytoreductive medications. Different approaches have been proposed by international disease experts for the diagnosis, thromboembolic risk estimation, and drug selection. This paper aims to review clinical aspects of PV and propose a management algorithm. The authors also point to still unresolved questions and unmet needs in diagnosis and management.
URI: https://doi.org/10.3906/sag-1806-43
https://journals.tubitak.gov.tr/medical/vol48/iss4/2/
http://hdl.handle.net/11452/34685
ISSN: 1300-0144
1303-6165
Koleksiyonlarda Görünür:Scopus
TrDizin
Web of Science

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