Polycythemia vera - PubMed
3 hours ago
- #thrombosis management
- #JAK2 mutation
- #myeloproliferative neoplasm
- Polycythemia vera (PV) is a myeloproliferative neoplasm caused by JAK2 mutations, leading to JAK-STAT pathway activation and panmyelosis.
- Common symptoms include erythrocytosis, leukocytosis, thrombocytosis, pruritus, headache, dizziness, and fatigue, with thromboembolism as a major complication.
- Diagnosis uses international criteria based on hematocrit levels, bone marrow morphology, JAK2 mutation status, and erythropoietin levels.
- Risk stratification for thrombosis relies on age and prior thrombosis history.
- Management focuses on maintaining hematocrit below 45% and preventing thrombosis via phlebotomy, aspirin, and cytoreductive agents like hydroxyurea.
- Newer treatments include ropeginterferon alfa-2b for hematologic remission and ruxolitinib for hydroxyurea-resistant cases, with novel agents targeting hepcidin and epigenetics under study.
- PV is currently incurable, and future research aims for early detection and disease-modifying treatments to improve outcomes.