Polycythemia vera (PV) is a rare blood cancer causing the bone marrow to make too many blood cells. Treatment usually starts with phlebotomy and may continue with medications. If one treatment isn’t effective, your doctor may revise it.

With PV, 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.

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

Usually, the first-line treatment for PV is a phlebotomy, which is a procedure that removes blood from your body. and temporarily reduces the concentration of red blood cells. Often, though not always, you will need to take a daily dose of aspirin while undergoing this treatment. This also helps thin your blood.

In addition, you may be treated in one or more of the following ways:

  • Anagrelide (Agrylin): This drug reduces platelets in your blood, which lowers the risk of a clot.
  • Antihistamines: These medications treat itchy skin, which is a common PV symptom.
  • Myelosuppressive drugs: The medications, like hydroxyurea, help reduce the amount of blood cells created in the bone marrow.
  • Ruxolitinib (Jakafi): This is a Janus Kinase (JAK) inhibitor that can help if your PV doesn’t respond to hydroxyurea, when hydroxyurea does not work well, or if you have an intermediate or high risk for myelofibrosis.
  • Interferon alfa: This drug reduces the production of blood cells but is rarely prescribed, as it tends to cause more side effects than other treatments.
  • Light therapy: This treatment uses psoralen and ultraviolet light can help relieve itchiness linked to PV.
  • Bone marrow transplant: This is sometimes used to reduce the number of blood cells in the bone marrow. It’s the only actual cure for PV and other blood cancers, but it comes with a high risk of complications.

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.

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.

If you are continuing to experience any of the following symptoms, or if these symptoms are worsening despite treatment, be sure to let your doctor know.

  • feeling fatigued or weak
  • headaches
  • dizziness
  • 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

Blood cancers like PV can sometimes be managed by just monitoring the symptoms. This applies to cases when you are not experiencing any symptoms.

People like this tend to be diagnosed accidentally through a blood test, but the disease isn’t necessarily affecting their quality of life.

Because the treatment focuses on managing symptoms, sometimes the best approach is to wait until there are symptoms to treat.

Q: When should you not “wait and watch” with polycythemia vera?

Anonymous

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.
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Excess blood cells in PV make blood thicker and more likely to clot. For this reason, thrombosis, or blood clots, is the most common complication of PV. Thrombosis can progress to more serious conditions, as outlined below, along with other possible complications.

Note the symptoms in this chart and get immediate care if you experience these.

ComplicationWhat it isSymptoms
deep vein thrombosis (DVT) blood clot in your veinSwelling, pain, cramping, and red or warm skin; This can progress to a heart attack or pulmonary embolism, which are life threatening.
pulmonary embolism (PV)blood clot in your lungsdifficulty breathing, chest pain, increased heart rate, bloody cough,
dizziness or fainting, low blood pressure

low oxygenslower blood flow, which reduces how much oxygen reaches your brain and organsfatigue and weakness, headache, dizziness, difficulty breathing, seeing flashes of light, chest pain, and ringing in your ears
bleedingtoo many plateletsbleeding easily, such as from your gums, nose, or in your stomach
pruritusPV triggers the release of histamine, the same chemical involved in allergic reactionsrash, itchiness
ulcershistamine makes your stomach release more acid, which causes peptic ulcersstomach pain, nausea, feeling bloated or full
enlarged spleenthe spleen works hard to remove excess blood cells, which causes it to growstomach pain, weight loss, swelling in the belly
goutPV causes excess uric acid, which can cause gout, a type of arthritis joint swelling, kidney stones
Other blood cancersPV can progress to myelofibrosis and acute myeloid leukemia (AML)similar symptoms to PV, but may not go away or may worsen.

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 chance of these complications by lowering the number of blood cells.

What is the life expectancy of polycythemia vera with and without treatment?

The median survival period for PV without treatment is 6-18 months after being diagnosed. With treatment, this increases to more than a decade.

Can diet and exercise help with polycythemia vera?

A healthy lifestyle can help support PV treatment. This includes eating a balanced diet and doing gentle exercises regularly.

Does drinking a lot of water help polycythemia?

Staying hydrated is always healthy, and it may help reduce the effects of complications such as gout and kidney stones.

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.