What Is Polycythemia Vera?

PV is a type of cancer that falls under the category of myeloproliferative neoplasms (MPNs). MPNs are “diseases in which your bone marrow is making too many blood cells,” according to Dr. Adrienne Phillips, a hematologist/oncologist at Weill Cornell Medicine.

• PV is more common in adults and can develop at any age.
• PV develops when an error occurs in the genes of your bone marrow cells. Most patients with PV have a genetic mutation in the JAK2 pathway that regulates blood cell production.
• If you had any prior radiation exposure, you may also be at risk.
• PV can run in your family. But most people with PV may not have a family history or genetic predisposition to this disease. You cannot catch PV from other people.

What Are The Symptoms of PV?

In the early stages of PV, you may not have any symptoms; a routine blood test at your doctor’s office may show an abnormal level of hemoglobin, white blood cells and/or platelets. Later in the course of the disease, you may feel a dull pain in your belly as your spleen grows. Many signs and symptoms occur because a large number of blood cells crowd out the bone marrow.

Common symptoms include:

• Frequent headaches
• Weakness/dizziness
• Night sweats
• Itching, especially after a warm bath or shower
• Painful, swollen joints, especially in the big toe, ankle, or knee
• Vision problems
• Stomach problems, such as pain in the upper belly

How Is PV Diagnosed?

PV is usually discovered incidentally at the doctor’s office during a routine blood test. Your doctor will confirm a diagnosis using the following tests:

• Complete blood count (CBC): This blood test looks at hemoglobin, different types of white blood cells, and platelets. In PV, your hemoglobin and red cell mass value are higher than normal along with a normal or high number of white blood cells and platelets.
• Serum Erythropoietin Test (EPO Test): This test checks for a hormone called erythropoietin (EPO). This hormone tells the bone marrow stem cells to make more red blood cells. This blood test is usually low in polycythemia vera.
• Blood smear: This test examines blood samples under a microscope to rule out other types of blood cancer such as acute leukemia.
• Bone marrow examination: Bone marrow examination may be needed in some patients to confirm the PV diagnosis. During a bone marrow biopsy, your doctor takes a small sample of your bone marrow, usually from the hip bone. Then the pathologist looks at the cells under a microscope to determine the presence of a mutation and/or chromosomal abnormality on the cancer cells.

How Is PV Treated?

Your doctor will determine your PV treatment based on your age and symptoms and whether you have a JAK mutation or blood clot. If you don’t have symptoms and aren’t experiencing blood clots, and if you are under 60 years old, then the treatment goal is to lower the number of red cells your body makes and to prevent blood clots and other complications.

“Patients with PV might need to have blood draws every month to make sure that their hemoglobin is not too high in preventing them from getting strokes,” Dr. Phillips says.

Treatment generally includes:

• Removing excess blood cells from your body by phlebotomy. This is similar to when you give blood. You may need to do this regularly to keep your red blood cell level close to normal.
• Your doctor may prescribe you a low-dose aspirin to prevent blood clots.
• Medicines such as hydroxyurea or interferon alfa (Intron A) can be also given by your doctor to keep your bone marrow from making too many red blood cells or platelets. Hydroxyurea is an oral chemotherapy medication that decreases the blood cells produced by your bone marrow. “Interferon alfa, which is a subcutaneous injection, and hydroxyurea, which is a chemotherapy pill, both of these drugs are effective at reducing the blood counts and bringing them down to normal and ideally preventing the need for doing phlebotomies in the future,” Dr. Ghaith Abu-Zeinah, an oncologist/hematologist at Weill Cornell Medicine, tells SurvivorNet.
• If hydroxyurea is not helpful in managing symptoms or causes too many side effects such as nausea, vomiting, diarrhea, leg ulcers, flu like symptoms, bone pain, and loss of appetite, your doctor may change the medicine to ruxolitinib (Jakafi)  to control the disease. Ruxolitinib is an FDA-approved chemotherapy drug that binds to and blocks JAK mutation; this can significantly improve your symptoms and blood count. You may experience bloating, dizziness, or headache, changes in blood count,an increased risk of infection and bleeding. You need to inform your doctor if you experience any of these side effects.
  

Are There Any New Treatments For PV?

BESREMi (ropeginterferon alfa-2b-njft) is a newly-approved injectable medication used to treat PV.

Unlike other treatments, BESREMi specifically targets the underlying disease mechanism.

It’s administered as an injection and works by boosting the immune system. Patients receive it once every two weeks or longer, allowing flexible dosing based on individual needs.

However, like any medication, it can have side effects, including:

• Fatigue
• Weakness
• Risk of infection.

Although it is a relatively new option, most insurance companies offer coverage for this drug. There are also financial assistance programs available to help patients access this treatment.

It’s worth a discussion with your doctor to see if BESREMi could be a good choice for you.

Questions to Ask Your Doctor

• Should members of family be tested for PV?
• What is the best way to manage my PV symptoms?
• Are there side effects to consider while treating PV?
• How do we monitor the progress of my PV?

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