Disease: Polycythemia vera

Overview

Polycythemia vera (pol-e-sy-THEE-me-uh VEER-uh) is a slow-growing blood cancer in which your bone marrow makes too many red blood cells. These excess cells thicken your blood, slowing its flow. They also cause complications, such as blood clots, which can lead to a heart attack or stroke.

Polycythemia vera isn't common. 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. Over time, in some cases there's a risk of progressing to more-serious blood cancers, such as myelofibrosis or acute leukemia.

Source: http://www.mayoclinic.com

Symptoms

Many people with polycythemia vera don't have signs or symptoms. Others might have:

  • Itchiness, especially following a warm bath or shower
  • Headache
  • Dizziness
  • Bleeding or bruising, usually minor
  • Weakness
  • Fatigue
  • Blurred vision
  • Excessive sweating
  • Painful swelling of one joint, often the big toe
  • Shortness of breath
  • Numbness, tingling, burning or weakness in your hands, feet, arms or legs
  • A feeling of fullness or bloating in your left upper abdomen due to an enlarged spleen
  • Fevers
  • Unexplained weight loss

When to see a doctor

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

Source: http://www.mayoclinic.com

Causes

Polycythemia vera is one of a group of blood cancers known as myeloproliferative neoplasms. It 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 blood cells.

The mutation that causes polycythemia vera is thought to affect a protein switch that tells the cells to grow. Specifically, it's a mutation in the protein Janus kinase 2 (JAK2). Most people with polycythemia vera have this mutation. The cause of the mutation isn't known, but it's generally not inherited.

Source: http://www.mayoclinic.com

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:

  • An increase in the number of red blood cells and, in some cases, an increase in platelets or white blood cells
  • Increased 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 you have polycythemia vera, he or she might recommend a bone marrow aspiration or biopsy to collect a sample of your bone marrow for study. 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.

Tests for the gene mutation that causes polycythemia vera

If you have polycythemia vera, analysis of your bone marrow or blood also might show the JAK2 mutation in the cells that's associated with the disease.

Source: http://www.mayoclinic.com

Complications

Possible complications of polycythemia vera include:

  • Blood clots. Increased blood thickness and decreased blood flow, as well as abnormalities in your platelets, increase your risk of blood clots. Blood clots can cause a stroke, a heart attack or a blockage of an artery in your lungs (pulmonary embolism) or in a vein deep within a muscle (deep vein thrombosis).
  • Enlarged spleen (splenomegaly). 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 (myelofibrosis), a condition in which stem cells don't mature or function properly (myelodysplastic syndrome), or cancer of the blood and bone marrow (acute leukemia).

Source: http://www.mayoclinic.com

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, which decreases 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.
  • 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.

Source: http://www.mayoclinic.com

Risk factors

Polycythemia vera can occur at any age, but it's more common in adults older than 60.

Source: http://www.mayoclinic.com

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