Polycythemia vera (PV) is a rare blood cancer where the bone marrow makes too many blood cells. Extra red blood cells make the blood thicker and increase the risk of a blood clot.
There’s no current cure for PV, but treatments can help prevent complications and address symptoms.
Your doctor will schedule regular tests and appointments to monitor your health. It’s important to regularly check in with your healthcare team so they know how you’re feeling.
Read on to find out more about how PV is managed, and how to know whether treatments are working.
PV tends to be found through routine blood work rather than experiencing symptoms. Many symptoms of PV have other causes, so they’re not always red flags on their own. Talk to your doctor if you notice any change in how you’re feeling.
If you do have symptoms, you may experience:
- feeling fatigued or weak
- ringing in the ears (tinnitus)
- reddened skin
- vision problems, including blind spots or blurry vision
- itchy skin, especially after a hot bath or shower
- abdominal pain or a feeling of fullness (resulting from an enlarged spleen)
- chest pain
- joint pain or swelling
Excess blood cells in PV make blood thicker and more likely to clot. This may lead to a potentially deadly heart attack, stroke, or pulmonary embolism linked to deep vein thrombosis.
While PV isn’t curable, that doesn’t mean it can’t be effectively managed for a very long time. PV treatments aim to reduce symptoms and decrease the risk of complications related to blood clots by lowering the number of blood cells.
Your healthcare team will discuss the best treatments for your PV depending on your blood levels and symptoms.
Your doctor may prescribe medications to:
- thin blood
- prevent complications
- manage symptoms
It’s important to take medications exactly as directed.
The following treatments are commonly used to treat PV:
- Phlebotomy, or removing blood from the body, temporarily reduces the concentration of red blood cells and thins your blood.
- Low-dose aspirin therapy helps thin your blood.
- Anagrelide (Agrylin) reduces platelets in your blood, which lowers the risk of a clot.
- Antihistamines treat itchy skin, a common PV symptom.
- Myelosuppressive drugs like hydroxyurea reduce the amount of blood cells created in bone marrow.
- Ruxolitinib (Jakafi) can help if your PV doesn’t respond to hydroxyurea, or if you have an intermediate or high risk for myelofibrosis.
- Interferon alfa reduces the production of blood cells but is rarely prescribed, as it tends to cause more side effects than other treatments.
- Light therapy using psoralen and ultraviolet light can help relieve itchiness linked to PV.
- Bone marrow transplants are sometimes used to reduce the number of blood cells in the bone marrow.
Q: When should you not “wait and watch” with polycythemia vera?
A: People with PV could experience serious complications from their disease, such as blood clots or bleeding.
You should be started on phlebotomy if your hematocrit — the percentage of red blood cells in your total blood volume — is consistently greater than 45% to reduce the risk of blood clots or other symptoms such as itching or headaches.
Treatment for PV is based on the risk stage, which is determined by three factors: age (older or younger than 60), history of blood clots, and the absence or presence of a JAK2 mutation in the blood or bone marrow.
If you’re at very low risk (no history of clots, younger than 60, and no JAK2 mutation), you can be observed if your hematocrit is consistently less than 45%. If you also have cardiovascular risk factors, then daily aspirin should be considered.
If you’re at low risk (no history of thrombosis, younger than 60, but with a JAK2 mutation), then you should at least be on daily aspirin. If you have cardiovascular risk factors, then aspirin twice per day is more beneficial.
If you’re at intermediate risk (older than 60, no history of thrombosis, and no JAK2 mutation), you should be started on a medication called hydroxyurea.
If you’re considered at high risk (history of blood clots, over the age of 60, and with a JAK2 mutation), you should be on hydroxyurea and possibly a blood thinner, given the previous history of blood clots.
A “wait and watch” approach for PV is suitable for people who are younger, without a history of clots or JAK2 mutation, and with hematocrit less than 45%.Sheel Patel, MDAnswers represent the opinions of our medical experts. All content is strictly informational and should not be considered medical advice.
PV is a chronic disease that can be successfully managed for many years. Working closely with your healthcare team ensures they’re aware of any changes in your health so they can adjust your treatment plan as needed.
Managing PV requires routine visits with a cancer specialist (oncologist) and a blood doctor (hematologist). These doctors will regularly monitor your blood cell levels to guide treatment decisions.
Be sure to let your healthcare providers know if you experience any new symptoms, such as abdominal pain or joint swelling.
Your current treatments may not be working if they don’t address symptoms, or if blood work shows abnormal levels of blood cells.
In this case, your doctor may adjust your PV treatment plan. This may involve changing the dose of your medications or trying a new treatment.
Polycythemia vera (PV) is a type of blood cancer that can thicken blood and increase the risk of clots. Careful monitoring and management can reduce symptoms and the risk of complications.
Management for PV includes regular blood work, and may include medications and phlebotomy. Keep in touch with your healthcare team and follow your treatment plan to feel your best.