From the Guidelines
Polycythemia vera symptoms are managed through a combination of phlebotomy and medications, with the primary goal of maintaining the hematocrit below 45% to reduce blood viscosity and prevent thrombotic events, as supported by the European LeukemiaNet recommendations 1. The treatment approach involves:
- Regular phlebotomy to maintain the hematocrit below 45%
- Low-dose aspirin (81-100 mg daily) to reduce the risk of blood clots
- Cytoreduction with hydroxyurea as the first-line medication, usually started at 500-1000 mg daily and adjusted based on blood counts
- Interferon-alpha (particularly pegylated forms like peginterferon alfa-2a at 45-180 mcg weekly) as an alternative, especially for younger patients or those planning pregnancy
- Ruxolitinib (starting at 10 mg twice daily) for patients with significant symptoms or those who don't respond to hydroxyurea, as recommended by the European LeukemiaNet 1 Key considerations include:
- Maintaining a hematocrit below 45% to reduce the risk of thrombotic events, as demonstrated by the CYTO-PV trial 1
- Monitoring blood counts regularly and adjusting treatment as needed
- Managing cardiovascular risk factors aggressively
- Advising patients to stop smoking and maintain a healthy lifestyle
- Considering the use of ruxolitinib for patients with significant symptoms or those who don't respond to hydroxyurea, as supported by the RESPONSE trial 1
From the Research
Polycythemia Vera Symptoms Management
The management of Polycythemia Vera (PV) symptoms involves a combination of treatments aimed at reducing the risk of thrombotic events and alleviating burdensome symptoms.
- Treatment with aspirin and hematocrit control with phlebotomy are recommended for all patients with PV 2.
- Patients with high-risk status or poor hematocrit control may benefit from cytoreductive therapy with hydroxyurea, although approximately 1 in 4 patients develops resistance or intolerance 2.
- For patients who are resistant to or intolerant of hydroxyurea, ruxolitinib, a Janus kinase 1/2 inhibitor, provides hematocrit control, reduces spleen size, normalizes blood counts, and improves PV-related symptoms 2, 3.
- Ruxolitinib is a treatment option for patients who fail frontline therapies, such as hydroxyurea or interferon, and can alleviate pruritus and decrease splenomegaly in patients who are intolerant of or resistant to hydroxyurea 3, 4.
- Anagrelide may be used in conjunction with hydroxyurea in patients with excessive thrombocytosis, and other treatment options, such as interferon alfa and ropeginterferon alfa-2b, may be considered for patients with specific needs or preferences 3, 5.
Treatment Options
The choice of treatment for PV depends on the individual patient's risk factors, symptoms, and response to therapy.
- Phlebotomy and low-dose aspirin are sufficient for low-risk patients, while cytoreductive therapies are indicated for high-risk patients 5.
- Hydroxyurea or interferon may be used as first-line treatments, with ruxolitinib and anagrelide considered as second-line options 3, 5.
- Other treatment options, such as busulfan, givinostat, and idasanutlin, may be considered for patients with limited life expectancy or those who have failed other therapies 5, 6.
Disease Management
PV is a myeloproliferative neoplasm characterized by erythrocytosis and is almost universally associated with a JAK2 gene variant 4.
- The disease is associated with an increased risk of arterial and venous thrombosis, hemorrhage, myelofibrosis, and acute myeloid leukemia 4.
- To decrease the risk of thrombosis, all patients with PV should be treated with aspirin and therapeutic phlebotomy to maintain a hematocrit of less than 45% 4.
- Cytoreductive therapies, such as hydroxyurea or interferon, are recommended for patients at high risk of thrombosis 4.