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What Is Polycythemia Vera?

By

Allie Lemco Toren

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This content is created or selected by the Healthgrades editorial team and is funded by an advertising sponsor. The content is subject to the Healthgrades medical review process for accuracy, balance and objectivity. The content is not edited or otherwise influenced by the advertisers appearing on this page except with the possible suggestion of the broad topic area. For more information, read the HealthGrades advertising policy.

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At Your Appointment

What to Ask Your Doctor About PV

Treating Rare Blood Disorders

New advances in treatment for myeloproliferative diseases offer hope for people with these rare blood cancers.
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Unfortunately, a lot of people today have experienced the fight against cancer ourselves or watched a loved one battle it—we’re all too familiar with the most common types. But there are many cancers that are far more rare than breast or lung cancer. One of those more rare cancers is called polycythemia vera (PV). PV falls under a group of blood cancers called myeloproliferative neoplasms (MPN). In patients with PV, the bone marrow produces too many red blood cells. Sometimes, PV can also cause your body to make too many of the other types of blood cells: white blood cells and platelets. When you have too many blood cells, this thickens your blood, which can cause complications like bleeding or blood clots. This is a serious problem, since blood clots that occur in your head can cause a stroke, and blood clots occurring in your arteries can cause a heart attack. And in some cases, these extra blood cells can group together in the spleen and cause it to enlarge. PV can be life-threatening if it goes untreated, but there’s good news: with the right medical care, many people live normal lives with few, if any, problems related to the condition.

Cause

PV occurs more commonly in men than women, and it rarely affects people younger than 60 years old. Experts believe that PV may be caused by a genetic mutation. Usually, your body regulates the number of blood cells you have, but 95% of patients with PV have a defect in a regulatory gene called JAK2 that interrupts the body’s careful balance. This defect leads to unchecked blood cell proliferation, yielding abnormalities in blood cell number and their functional quality. Researchers don’t know what causes this genetic mutation, but they don’t believe it’s hereditary.

It’s also possible that exposure to intense amounts of radiation and possibly toxic substances may increase the risk of PV, but this theory is new and requires more data to back it up.

Symptoms

In many cases, polycythemia vera doesn’t cause any signs or symptoms, but some people may experience:

  • Headache

  • Sweating, especially at night

  • Ringing in the ears

  • Blurred visions

  • Dizziness or vertigo

  • Reddish or purplish skin

  • Bleeding or clothing

  • Unexpected weight loss

  • Feeling very full or bloated (caused by an enlargement of your spleen)

  • Itching, especially after a shower

  • Burning, red hands and feet

  • Fatigue

  • Painful swelling of one joint, especially the big toe

  • Difficulty breathing when lying down

Some people experience all these symptoms, just several, or none at all.

Diagnosis

Since PV doesn’t always show clear symptoms, many patients are diagnosed after a routine blood test reveals an increase in their red blood cells, sometimes along with an increase in white blood cells or platelets. But patients may also get diagnosed after seeing their doctors about their symptoms. There are several diagnostic tests that doctors use to see if someone may have PV, including:

  • Blood tests: a complete blood count (CBC) can show an increase in the blood cell levels in your body.

  • Bone marrow aspiration or biopsy: this procedure can examine if there’s a problem with how the bone marrow is producing blood cells.

  • Test for genetic mutations: since almost all PV patients have a JAK2 gene mutation, doctors may test to see if the mutation is present.

  • Low erythropoietin level testing: erythropoietin is a hormone involved in the production of red blood cells. If there are low levels of this hormone, that may be a sign of PV.

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Medical Reviewers: William C. Lloyd III, MD, FACS Last Review Date: Jun 20, 2016

© 2016 Healthgrades Operating Company, Inc. All rights reserved. May not be reproduced or reprinted without permission from Healthgrades Operating Company, Inc. Use of this information is governed by the Healthgrades User Agreement.

View Sources

Medical References

  1. Polycythemia Vera. MedlinePlus, U.S. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/000589.htm
  2. Polycythemia Vera. Mayo Clinic. http://www.mayoclinic.org/diseases-conditions/polycythemia-vera/basics/definition/con-20031013
  3. Polycythemia Vera (PV). MPN (Myeloproliferative Neoplasms) Research Foundation. http://www.mpnresearchfoundation.org/Polycythemia-Vera-28PV-29
  4. Chronic Myeloproliferative Neoplasms Treatment (PDQ)—Patient Version. National Cancer Institute. http://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq
  5. Definition: Polycythemia Vera. National Cancer Institute. http://www.cancer.gov/common/popUps/popDefinition.aspx?id=CDR0000426418&version=Patient&language=English

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