Polycythemia vera (PV) doesn't have a cure. However, treatments can help control the disease and its complications. PV is treated with procedures, medicines, and other methods. You may need one or more treatments to manage the disease.
Goals of Treatment
The goals of treating PV are to control symptoms and reduce the risk of complications, especially heart attack and stroke. To do this, PV treatments reduce the number of red blood cells and the level of hemoglobin (an iron-rich protein) in the blood. This brings the thickness of your blood closer to normal.
Blood with normal thickness flows better through the blood vessels. This reduces the chance that blood clots will form and cause a heart attack or stroke.
Blood with normal thickness also ensures that your body gets enough oxygen. This can help reduce some of the signs and symptoms of PV, such as headaches, vision problems, and itching.
Studies show that treating PV greatly improves your chances of living longer.
The goal of treating secondary polycythemia is to control its underlying cause, if possible. For example, if the cause is carbon monoxide exposure, the goal is to find the source of the carbon monoxide and fix or remove it.
Treatments To Lower Red Blood Cell Levels
Phlebotomy
Phlebotomy (fle-BOT-o-me) is a procedure that removes some blood from your body. For this procedure, a needle is inserted into one of your veins. Blood from the vein flows through an airtight tube into a sterile container or bag. The process is similar to the process of donating blood.
Phlebotomy reduces your red blood cell count and starts to bring your blood thickness closer to normal.
Typically, a pint (1 unit) of blood is removed each week until your hematocrit level approaches normal. (Hematocrit is the measure of how much space red blood cells take up in your blood.)
You may need to have phlebotomy done every few months.
Medicines
Your doctor may prescribe medicines to keep your bone marrow from making too many red blood cells. Examples of these medicines include hydroxyurea and interferon-alpha.
Hydroxyurea is a medicine generally used to treat cancer. This medicine can reduce the number of red blood cells and platelets in your blood. As a result, this medicine helps improve your blood flow and bring the thickness of your blood closer to normal.
Interferon-alpha is a substance that your body normally makes. It also can be used to treat PV. Interferon-alpha can prompt your immune system to fight overactive bone marrow cells. This helps lower your red blood cell count and keep your blood flow and blood thickness closer to normal.
Radiation Treatment
Radiation treatment can help suppress overactive bone marrow cells. This helps lower your red blood cell count and keep your blood flow and blood thickness closer to normal.
However, radiation treatment can raise your risk of leukemia (blood cancer) and other blood diseases.
Treatments for Symptoms
Aspirin can relieve bone pain and burning feelings in your hands or feet that you may have as a result of PV. Aspirin also thins your blood, so it reduces the risk of blood clots.
Aspirin can have side effects, including bleeding in the stomach and intestines. For this reason, take aspirin only as your doctor recommends.
If your PV causes itching, your doctor may prescribe medicines to ease the discomfort. Your doctor also may prescribe ultraviolet light treatment to help relieve your itching.
Other ways to reduce itching include:
- Avoiding hot baths. Cooler water can limit irritation to your skin.
- Gently patting yourself dry after bathing. Vigorous rubbing with a towel can irritate your skin.
- Taking starch baths. Add half a box of starch to a tub of lukewarm water. This can help soothe your skin.
Experimental Treatments
Researchers are studying other treatments for PV. An experimental treatment for itching involves taking low doses of selective serotonin reuptake inhibitors (SSRIs). This type of medicine is used to treat depression. In clinical trials, SSRIs reduced itching in people who had PV.
Imatinib mesylate is a medicine that's approved for treating leukemia. In clinical trials, this medicine helped reduce the need for phlebotomy in people who had PV. This medicine also helped reduce the size of enlarged spleens.
Researchers also are trying to find a treatment that can block or limit the effects of an abnormal JAK2 gene. (A mutation, or change, in the JAK2 gene is the major cause of PV.)
Source: National Heart, Lung, and Blood Institute, National Institutes of Health.
Diagnosis
Your doctor will take a detailed medical history and perform a physical exam.
Blood tests
If you have polycythemia vera, blood tests might reveal:
- More red blood cells than normal and, sometimes, an increase in platelets or white blood cells
- A greater percentage of red blood cells that make up total blood volume (hematocrit measurement)
- Elevated levels of the iron-rich protein in red blood cells that carries oxygen (hemoglobin)
Bone marrow aspiration or biopsy
If your doctor suspects that you have polycythemia vera, he or she might recommend collecting a sample of your bone marrow through a bone marrow aspiration or biopsy.
A bone marrow biopsy involves taking a sample of solid bone marrow material. A bone marrow aspiration is usually done at the same time. During an aspiration, your doctor withdraws a sample of the liquid portion of your marrow.
Specific gene testing
If you have polycythemia vera, analysis of your bone marrow or blood might show the gene mutation that's associated with the disease.
Treatment
There's no cure for polycythemia vera. Treatment focuses on reducing your risk of complications. These treatments may also ease your symptoms.
Blood withdrawals
The most common treatment for polychythemia vera is having frequent blood withdrawals, using a needle in a vein (phlebotomy). It's the same procedure used for donating blood.
This decreases your blood volume and reduces the number of excess blood cells. How often you need to have blood drawn depends on the severity of your condition.
Treatments to reduce itching
If you have bothersome itching, your doctor may prescribe medication, such as antihistamines, or recommend ultraviolet light treatment to relieve your discomfort.
Medications that are normally used to treat depression, called selective serotonin reuptake inhibitors (SSRIs), helped relieve itching in clinical trials. Examples of SSRIs include paroxetine (Brisdelle, Paxil, Pexeva, others) or fluoxetine (Prozac, Sarafem, Selfemra, others).
Drugs that reduce the number of red blood cells
If phlebotomy alone doesn't help enough, your doctor may suggest medications that can reduce the number of red blood cells in your bloodstream. Examples include:
- Hydroxyruea (Droxia, Hydrea)
- Interferon alfa-2b (Intron A)
- Ruxolitinib (Jakafi)
- Busulfan (Busulfex, Myleran)
Heart medications
Your doctor will also likely prescribe medications to control risk factors for heart and blood vessel disease, including high blood pressure, diabetes and abnormal cholesterol.
Your doctor may recommend that you take a low dose of aspirin to reduce your risk of blood clots. Low-dose aspirin may also help reduce burning pain in your feet or hands.
Clinical trials
Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this condition.
Lifestyle and home remedies
You can take steps to help yourself feel better if you've been diagnosed with polycythemia vera. Try to:
- Exercise. Moderate exercise, such as walking, can improve your blood flow. This helps decrease your risk of blood clots. Leg and ankle stretches and exercises also can improve your blood circulation.
- Avoid tobacco. Using tobacco can cause your blood vessels to narrow, increasing the risk of heart attack or stroke due to blood clots.
- Avoid low-oxygen environments. Living at high altitudes, skiing or climbing in mountains all reduce the oxygen levels in your blood even further.
Be good to your skin. To reduce itching, bathe in cool water, use a gentle cleanser and pat your skin dry. Adding starch, such as cornstarch, to your bath might help. Avoid hot tubs, heated whirlpools, and hot showers or baths.
Try not to scratch, as it can damage your skin and increase the risk of infection. Use lotion to keep your skin moist.
- Avoid extreme temperatures. Poor blood flow increases your risk of injury from hot and cold temperatures. In cold weather, always wear warm clothing, particularly on your hands and feet. In hot weather, protect yourself from the sun and drink plenty of liquids.
- Watch for sores. Poor circulation can make it difficult for sores to heal, particularly on your hands and feet. Inspect your feet regularly and tell your doctor about any sores.
Preparing for your appointment
You're likely to start by seeing your primary care physician. If you're diagnosed with polycythemia vera, you might be referred to a doctor who specializes in blood conditions (hematologist).
Here's some information to help you get ready for your appointment.
What you can do
Make a list of:
- Your symptoms, including any that seem unrelated to the reason for which you scheduled the appointment, and when they began
- Key personal information, including other medical conditions and family medical history
- All medications, vitamins or supplements you take, including doses
- Questions to ask your doctor
For polycythemia vera, questions to ask your doctor include:
- What's the most likely cause of my symptoms?
- What tests do I need?
- Is this condition temporary, or will I always have it?
- What treatments are available, and which do you recommend?
- I have other health conditions. How can I best manage them together?
- Should I see a specialist?
- Will I need follow-up visits? If so, how often?
- Are there brochures or other printed material I can have? What websites do you recommend?
Don't hesitate to ask other questions you think of during the appointment. Take a family member or friend along, if possible, to help you remember the information you're given.
What to expect from your doctor
Your doctor is likely to ask you questions, including:
- Have your symptoms been continuous or occasional?
- How severe are your symptoms?
- What, if anything, seems to improve your symptoms?
- Does anything worsen your symptoms?
Feb. 11, 2022