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Polycythemia vera

Overview

Polycythemia vera (pol-e-sy-THEE-me-uh VEER-uh) is a type of blood cancer. It causes your bone marrow to make too many red blood cells. These excess cells thicken your blood, slowing its flow, which may cause serious problems, such as blood clots.

Polycythemia vera is rare. It usually develops slowly, and you might have it for years without knowing. Often the condition is found during a blood test done for another reason.

Without treatment, polycythemia vera can be life-threatening. But proper medical care can help ease signs, symptoms and complications of this disease.

Symptoms

Many people with polycythemia vera don't have noticeable signs or symptoms. Some people might develop vague symptoms such as headache, dizziness, fatigue and blurred vision.

More-specific symptoms of polycythemia vera include:

  • Itchiness, especially after a warm bath or shower
  • Numbness, tingling, burning, or weakness in your hands, feet, arms or legs
  • A feeling of fullness soon after eating and bloating or pain in your left upper abdomen due to an enlarged spleen
  • Unusual bleeding, such as a nosebleed or bleeding gums
  • Painful swelling of one joint, often the big toe
  • Shortness of breath and difficulty breathing when lying down

When to see a doctor

Make an appointment with your doctor if you have signs or symptoms of polycythemia vera.

Causes

Polycythemia vera occurs when a mutation in a gene causes a problem with blood cell production. Normally, your body regulates the number of each of the three types of blood cells you have — red blood cells, white blood cells and platelets. But in polycythemia vera, your bone marrow makes too many of some of these blood cells.

The cause of the gene mutation in polycythemia vera is unknown, but it's generally not inherited from your parents.

Risk factors

Polycythemia vera can occur at any age, but it's more common in adults between 50 and 75. Men are more likely to get polycythemia vera, but women tend to get the disease at younger ages.

Complications

Possible complications of polycythemia vera include:

  • Blood clots. Increased blood thickness and decreased blood flow, as well as abnormalities in your platelets, raise your risk of blood clots. Blood clots can cause a stroke, a heart attack, or a blockage in an artery in your lungs or a vein deep within a leg muscle or in the abdomen.
  • Enlarged spleen. Your spleen helps your body fight infection and filter unwanted material, such as old or damaged blood cells. The increased number of blood cells caused by polycythemia vera makes your spleen work harder than normal, which causes it to enlarge.
  • Problems due to high levels of red blood cells. Too many red blood cells can lead to a number of other complications, including open sores on the inside lining of your stomach, upper small intestine or esophagus (peptic ulcers) and inflammation in your joints (gout).
  • Other blood disorders. In rare cases, polycythemia vera can lead to other blood diseases, including a progressive disorder in which bone marrow is replaced with scar tissue, a condition in which stem cells don't mature or function properly, or cancer of the blood and bone marrow (acute leukemia).

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)
  • Very low levels of a hormone that stimulates bone marrow to produce new red blood cells (erythropoietin)

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.

A needle suctioning out liquid bone marrow from hipbone

In a bone marrow aspiration, a doctor or nurse uses a thin needle to remove a small amount of liquid bone marrow, usually from a spot in the back of your hipbone (pelvis). A bone marrow biopsy is often done at the same time. This second procedure removes a small piece of bone tissue and the enclosed marrow.

Treatment

There's no cure for polycythemia vera. Treatment focuses on reducing your risk of complications. These treatments may also ease your symptoms.

Treatment to reduce symptoms might include:

  • Low-dose aspirin. 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.
  • Therapy 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).

Treatments to reduce the amount of red blood cells include:

  • Reducing the amount of blood in your body. Drawing some blood out of your veins in a procedure called phlebotomy is usually the main treatment for people with polycythemia vera. 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.
  • Medication to decrease blood cells. If phlebotomy alone doesn't help, medications that suppress your bone marrow's ability to produce blood cells, such as hydroxyurea (Droxia, Hydrea), might be used.

    Interferon alfa-2b (Intron A) may be used to stimulate your immune system to fight the overproduction of red blood cells. It might help people who don't respond well to hydroxyurea.

  • Medication to destroy cancer cells. Ruxolitinib (Jakafi) is approved by the Food and Drug Administration to treat people with polycythemia vera who don't respond to or can't take hydroxyurea. It helps your immune system destroy cancer cells, and can improve some polycythemia vera symptoms.

    Busulfan (Busulfex, Myleran) is another medication that destroys cancer cells that may be prescribed if other treatments aren't working well.

Researchers are studying drugs that inhibit a gene linked to polycythemia vera and other treatments.

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.

You'll need to see your doctor on an ongoing basis to monitor how well your current treatment is working. Ask your doctor how often you need to schedule follow-up appointments.

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 an 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?

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