Learn About Polycythemia Vera

What is the definition of Polycythemia Vera?

Polycythemia vera (PV) is a bone marrow disease that leads to an abnormal increase in the number of blood cells. The red blood cells are mostly affected.

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What are the alternative names for Polycythemia Vera?

Primary polycythemia; Polycythemia rubra vera; Myeloproliferative disorder; Erythremia; Splenomegalic polycythemia; Vaquez's disease; Osler's disease; Polycythemia with chronic cyanosis; Erythrocytosis megalosplenica; Cryptogenic polycythemia

What are the causes of Polycythemia Vera?

PV is a disorder of the bone marrow. It mainly causes too many red blood cells to be produced. The numbers of white blood cells and platelets may also be higher than normal.

PV is a rare disorder that occurs more often in men than in women. It is not usually seen in people under age 40. The problem is often linked to a gene defect called JAK2V617F. The cause of this gene defect is unknown. This gene defect is not an inherited disorder.

What are the symptoms of Polycythemia Vera?

With PV, there are too many red blood cells in the body. This results in very thick blood, which can't flow through small blood vessels normally, leading to symptoms such as:

  • Trouble breathing when lying down
  • Dizziness
  • Feeling tired all the time
  • Full feeling in the left upper abdomen (due to enlarged spleen)
  • Headache
  • Itchiness, especially after a warm bath
  • Red skin coloring, especially of the face
  • Shortness of breath
  • Symptoms of blood clots in veins near the skin surface (phlebitis)
  • Vision problems
  • Ringing in the ears (tinnitus)
  • Joint pain
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What are the current treatments for Polycythemia Vera?

The goal of treatment is to reduce the thickness of the blood and prevent bleeding and clotting problems.

A method called phlebotomy is used to decrease blood thickness. One unit of blood (about 1 pint, or 1/2 liter) is removed each week until the number of red blood cells drops. The treatment is continued as needed.

Medicines that may be used include:

  • Hydroxyurea to reduce the number of red blood cells made by the bone marrow. This drug may be used when the numbers of other blood cell types are also high.
  • Interferon to lower blood counts.
  • Anagrelide to lower platelet counts.
  • Ruxolitinib (Jakafi) to reduce the number of red blood cells and reduce an enlarged spleen. This drug is prescribed when hydroxyurea and other treatments have failed.

Taking aspirin to reduce the risk of blood clots may be an option for some people, but aspirin increases the risk for stomach bleeding.

Ultraviolet-B light therapy can reduce the severe itching some people experience.

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What are the support groups for Polycythemia Vera?

More information and support for people with polycythemia vera and their families can be found at:

  • Leukemia and Lymphoma Society -- www.lls.org/myeloproliferative-neoplasms/polycythemia-vera
  • MPN Research Foundation -- www.mpnresearchfoundation.org/living-with-an-mpn/
  • National Organization for Rare Disorders -- rarediseases.org/rare-diseases/polycythemia-vera/
  • NIH Genetic and Rare Diseases Information Center -- rarediseases.info.nih.gov/diseases/7422/polycythemia-vera
What is the outlook (prognosis) for Polycythemia Vera?

PV usually develops slowly. Most people do not have symptoms related to the disease at the time of diagnosis. The condition is often diagnosed before severe symptoms occur.

What are the possible complications of Polycythemia Vera?

Complications of PV may include:

  • Acute myelogenous leukemia (AML)
  • Bleeding from the stomach or other parts of the intestinal tract
  • Gout (painful swelling of a joint)
  • Heart failure
  • Myelofibrosis (disorder of the bone marrow in which the marrow is replaced by fibrous scar tissue)
  • Thrombosis (blood clotting, which can cause a stroke, heart attack, or other body damage)
When should I contact a medical professional for Polycythemia Vera?

Call your provider if symptoms of PV develop.

What are the latest Polycythemia Vera Clinical Trials?
A Phase III, Randomised, Open-label, Multicenter International Trial Comparing Ruxolitinib With Either HydRoxycarbamIDe or Interferon Alpha as First Line ThErapy for High Risk Polycythemia Vera
Summary: The trial will be a phase III, randomised-controlled, multi-centre, international, open-label trial consisting of ruxolitinib versus best available therapy, where best available therapy is a choice of interferon alpha, any formulation permitted (IFN) or hydroxycarbamide (HC), and which will be elected by the Investigator prior to randomisation.
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Characteristic of Patients With Gastroesophageal Varices and Portal Cavernoma
Summary: Myeloproliferative neoplasms (MPNs), including polycythemia vera, essential thrombocythemia, and primary myelofibrosis, may lead to gastroesophageal varices. The quality of life, morbidity, and mortality of MPN patients mainly depend on disease-related symptoms, thromboembolic and hemorrhagic complications. Previous studies have shown that JAK2 V617F has a prominent role in vascular risk and MPN-a...
What are the Latest Advances for Polycythemia Vera?
Anagrelide for platelet-directed cytoreduction in polycythemia vera: Insights into utility and safety outcomes from a large multi-center database.
Summary: Anagrelide for platelet-directed cytoreduction in polycythemia vera: Insights into utility and safety outcomes from a large multi-center database.
Ruxolitinib versus best available therapy in inadequately controlled polycythaemia vera without splenomegaly (RESPONSE-2): 5-year follow up of a randomised, phase 3b study.
Summary: Ruxolitinib versus best available therapy in inadequately controlled polycythaemia vera without splenomegaly (RESPONSE-2): 5-year follow up of a randomised, phase 3b study.
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Efficacy and safety of ropeginterferon alfa-2b in Japanese patients with polycythemia vera: an open-label, single-arm, phase 2 study.
Summary: Efficacy and safety of ropeginterferon alfa-2b in Japanese patients with polycythemia vera: an open-label, single-arm, phase 2 study.
Who are the sources who wrote this article ?

Published Date: January 19, 2021
Published By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Gotlib J. Polycythemia vera, essential thrombocythemia, and primary myelofibrosis. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 157.

Kremyanskaya M, Najfeld V, Mascarenhas J, Hoffman R. The polycythemias. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 68.

National Cancer Institute website. Chronic myeloproliferative neoplasms treatment (PDQ) -- health professional version. www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#link/. Updated November 4, 2020. Accessed March 25, 2021.