From the Guidelines
Polycythemia vera patients should be managed with phlebotomy to maintain the hematocrit at less than 45% and low-dose aspirin, with cytoreduction indicated in high-risk patients. The goal of treatment is to reduce the risk of thrombosis, strokes, and heart attacks, while improving quality of life and reducing morbidity and mortality.
Key Treatment Strategies
- Phlebotomy: to reduce blood thickness and maintain hematocrit below 45% 1
- Low-dose aspirin: to reduce clot risk, with a daily dose of 81-100 mg 1
- Cytoreductive therapy: for higher-risk patients, with options including hydroxyurea or interferon-alpha 1
High-Risk Patients
High-risk patients, defined as those with a history of previous thrombosis or age greater than 60 years, require cytoreductive therapy to reduce the risk of vascular events. Hydroxyurea and interferon-alpha are recommended as first-line treatments for high-risk patients 1.
Monitoring and Follow-up
Regular medical follow-up with complete blood counts is essential to monitor disease progression and adjust treatment as needed. Patients should also be advised to stay well-hydrated, avoid extreme temperatures, and manage their cardiovascular risk factors to reduce the risk of complications.
Recent Evidence
A recent study published in 2022 found that targeting hematocrit levels to <45% can lower the risk of thrombosis in patients with polycythemia vera 1. Another study published in 2015 recommended that hydroxyurea and interferon-alpha be used as first-line treatments for high-risk patients 1.
Treatment Goals
The primary goal of treatment is to reduce the risk of thrombosis, strokes, and heart attacks, while improving quality of life and reducing morbidity and mortality. By following these treatment strategies and monitoring patients closely, healthcare providers can help patients with polycythemia vera manage their disease and reduce the risk of complications.
From the Research
Definition and Diagnosis of Polycythemia Vera
- Polycythemia vera (PV) is a myeloproliferative neoplasm characterized by an increased red blood cell mass and increased risk of thrombosis 2.
- Erythrocytosis (hemoglobin >16.5 mg/dL in men or >16.0 mg/dL in women) is a required diagnostic criterion, although thrombocytosis (53%) and leukocytosis (49%) are common 2.
- More than 95% of patients have a JAK2 gene variant, which helps distinguish PV from secondary causes of erythrocytosis, such as tobacco smoking or sleep apnea 2.
Symptoms and Complications of Polycythemia Vera
- Patients may have pruritus (33%), erythromelalgia (5.3%), transient visual changes (14%), and splenomegaly (36%) with abdominal discomfort 2.
- PV is associated with an increased risk of arterial and venous thrombosis, hemorrhage, myelofibrosis, and acute myeloid leukemia 2, 3.
- The median survival from diagnosis is 14.1 to 27.6 years, and prior to or at the time of PV diagnosis, arterial thrombosis occurred in 16% of patients and 7% had venous thrombotic events 2.
Treatment and Management of Polycythemia Vera
- All patients with PV should receive therapeutic phlebotomy (goal hematocrit, <45%) and low-dose aspirin (if no contraindications) 2, 4, 5.
- Patients who are at higher risk of thrombosis include those aged 60 years or older or with a prior thrombosis, and these patients may benefit from cytoreductive therapy with hydroxyurea or interferon to lower thrombosis risk and decrease symptoms 2, 3, 4.
- Ruxolitinib is a Janus kinase inhibitor that can alleviate pruritus and decrease splenomegaly in patients who are intolerant of or resistant to hydroxyurea 2, 3.
- Phlebotomy and hydroxyurea are associated with improved overall survival and decreased risk of thrombosis in older adults with PV 5.
- Interferon-alpha is a preferred first-line agent for cytoreductive therapy due to improved survival outcomes 6.