Treating Polycythemia Vera

How Does Polycythemia Vera Progress?

Polycythemia vera, also called “PV,” is a chronic blood disorder in which a person’s bone marrow (the spongy tissue located in the center of your bones) makes too many red blood cells. People with PV also make too many white blood cells (the cells that fight infection) and platelets (the cells that help stop bleeding by forming clots).

A mutation of the JAK2 gene causes PV. The JAK2 gene creates a protein that helps your bone marrow make blood cells, and a change in the gene (called a mutation) leads to increased blood cell counts.

Experts are not sure why some people develop this mutation. While PV is not inherited (meaning the mutated gene is not passed down from a parent to his or her child), research suggests that some people have a genetic vulnerability to developing PV. In other words, for some unknown reason, certain people are more likely than others to acquire the JAK2 mutation.

PV's Effects

Red blood cells carry oxygen to the rest of your body via the bloodstream. When there are too many of them, your blood becomes thicker than normal, as shown.

Since thicker blood flows more slowly, a person with PV is at risk of not getting sufficient oxygen to their organs, causing various symptoms. Luckily, the right treatment can manage these as well as PV progression.

Discussing Treatments

Although PV isn’t curable, there are multiple treatment options available for stalling its progression and managing symptoms for a long time. Understanding why your doctor recommends a specific one is an empowering way to play an active role in your care.

If you're not sure where to start the conversation, this doctor discussion guide can help. Download it for a refresher on PV-related vocabulary and use the commonly asked questions to learn as much as you can about living with polycythemia vera.

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There are several categories of therapies available. In addition to taking them as prescribed, healthy diet, exercise, seeing your primary care doctor for preventive health care, and managing cardiovascular factors like diabetes or obesity are also essential.

Aspirin

The vast majority of people with PV take a low dose of aspirin everyday. The purpose of daily aspirin is to prevent blood clots. It can also alleviate symptoms of headache, vision disturbances, and burning hands and feet
Phlebotomy

Phlebotomy is a procedure in which blood is removed from your body, similar to what happens when you donate blood. The purpose of phlebotomy is to lower your hematocrit. It will ultimately thin out your blood, which will help prevent the formation of blood clots.
Droxia, Hydrea
(Hydroxyurea)

Droxia and Hydrea (hydroxyureas) are chemotherapy drugs taken by mouth. They reduce red blood cells and platelets in your bloodstream to help blood flow more easily.
Jakafi
(ruxolitinib)

Jakafi, a JAK1 and 2 enzyme inhibitor, is FDA-approved for those who do not respond well to or cannot tolerate hydroxyurea. It reduces hematocrit and may reduce spleen size and symptoms.
Pegasys (pegylated
interferon alpha-2a)

Pegasys triggers your own immune system to attack overactive bone marrow cells. It’s used “off-label” most often in younger people, especially women of childbearing age.
Busulfex, Myleran
(busulfan)

Because long-term use is associated with higher risk of leukemia transformation, busulfan is typically reserved for older individuals or people who cannot tolerate hydroxyurea or interferon-alpha.

Managing Signs and Symptoms

PV symptoms result primarily from an elevated red blood cell count. That said, a high number of platelets and white blood cells also play a role in certain symptoms and complications.

It’s important to note that the course of PV is quite variable, meaning some people have a mild disease with few symptoms whereas others have a more severe, debilitating disease. Those with few symptoms of PV are often only diagnosed incidentally after a routine medical visit.

And while nonspecific symptoms like headache, dizziness, facial flushing, weakness, excessive sweating, and unusual tiredness are common, there are also specific symptoms to note, along with helpful strategies for managing them.

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Itching

Itching, especially after a warm bath or shower, is due to elevated histamine levels. The severity varies from being mildly uncomfortable to utterly unbearable. It can occur anywhere, most commonly on the chest, back, middle of the arms, and the front of the legs, but you won’t see any skin rash or skin changes.

Coping with itching usually starts with simple remedies, then medications:
- Avoid triggers: Take cool showers instead of hot ones and wear loose, light pajamas at night.
- Hydrate your skin: Use delicate techniques such as applying moisturizers and patting your skin dry instead of rubbing.
- Ask your doctor about medications: Antihistamines (Benadryl or Allegra), low-dose aspirin, or an antidepressant may help.
- Explore other therapies: Therapies that lower the red blood cell may improve the sensation. Ultraviolet light therapy may also help.
Burning Hands and Feet

A burning sensation in the hands and feet is called erythromelalgia. It is caused by the aggregation of a high number of platelets in the bloodstream and is associated with a change in color of the hands and feet to white, blue, or red.

The good news is that erythromelalgia is soothed significantly by taking a low dose of aspirin.
Vision Changes

Vision problems ranging from blurry or double vision to transient blindness may occur in PV. They are a result of delayed blood flow to the retina, since the blood of people with PV is thick and moves sluggishly and slowly.

If you or a loved one has PV and are experiencing vision changes, especially if you have transient blindness, be sure to contact your oncologist right away. Early treatment with therapies that reduce the number of red blood cells is important to prevent possible permanent eye damage.
Gastrointestinal Disturbances

Left-sided abdominal discomfort and early satiety from an enlarged spleen (an organ that filters red blood cells and stores white blood cells and platelets) is common in PV. As a result of early satiety (and the disease process itself), weight loss may also occur. In addition, PV is associated with peptic ulcer disease due to elevated histamine levels in the body and increased gastric acidity.

Your doctor may offer solutions for your specific discomfort. Strategies like eating smaller meals more often can also help.
Blood Clots

Due to the increased thickness of the blood, a person with PV has an increased risk of developing a blood clot in an artery or vein. In other words, a person with PV has a higher chance than someone without PV (who is otherwise healthy) of having a stroke, heart attack, deep vein thrombosis (usually in the leg), or pulmonary embolus (blood clot in the lung). Clots may also occur in blood vessels in the abdomen, like those that supply the spleen and liver.

Blood clots are a very serious, potentially life-threatening complication of PV. To reduce your risk of developing them, a blood cancer doctor (oncologist) will recommend a daily dose of aspirin as well as phlebotomy (and maybe other therapies) to reduce the thickness of your blood.
Bleeding

The cause of bleeding in PV is acquired von Willebrand syndrome, which means that a defective von Willebrand factor (VWF) is absorbed onto the platelets. Since VWF is a protein that is essential to the clotting process, a defective protein means that bleeding may arise, especially in the gums and from small cuts.

Reducing the platelet count can treat the bleeding and this syndrome.