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Diseases

Genetic and Rare Diseases Information Center (GARD)

Polycythemia vera


Other Names for this Disease
  • Acquired primary erythocytosis
  • Erythremia
  • Osler-Vaquez disease
  • Polycythemia rubra vera
  • Primary polycythemia
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Treatment

Newline Maker

What treatments are available for itching related to polycythemia vera?

There are several treatments for the itching (pruritus) related to polycythemia vera (PV).  No single treatment has been found to be effective for all affected individuals.  For mild cases, treatment may include avoiding triggers of itching and dry skin, or controlling the temperature of the environment and bathing water.[1]  Several other treatments are available for more severe itching or for itching the does not respond to initial treatments.  Interferon-alpha has been found to be effective for reducing itching in a majority of individual with PV who received this therapy[2]; however, this medication can have significant side effects.[3]  Selective serotonin reuptake inhibitors (SSRIs), typically used to treat depression, may reducing itching for some individuals with PV.[2][3][1].  Phototherapy, antihistamines, and phlebotomy have also been attempted, with mixed results.[2][3][1]  Additionally, if a genetic cause of polycythemia vera is know, medications targeted to the causative gene - such as JAK or mTor inhibitors - may be helpful in reducing itching.[2][1]
Last updated: 9/7/2014

References
  1. Tefferi A. Polycythemia vera and essential thrombocythemia: 2012 update on diagnosis, risk stratification, and management. American Journal of Hematology. 2012; 87(3):285-293. http://www.ncbi.nlm.nih.gov/pubmed/22331582. Accessed 9/7/2014.
  2. Saini KS, Patnaik MM, Tefferi A. Polycythemia vera-associated pruritus and its management. European Journal of Clinical Investigation. 2010; 40(9):828-834. http://www.ncbi.nlm.nih.gov/pubmed/20597963. Accessed 9/7/2014.
  3. Tefferi A, Fonseca R. Selective serotonin reuptake inhibitors are effective in the treatment of polycythemia vera-associated pruritus. Blood. 2002; 99(7):2627. http://www.ncbi.nlm.nih.gov/pubmed/11926187. Accessed 9/7/2014.


GARD Video Tutorial

  • Finding Treatment Information - A video developed by GARD Information Specialists that explains how you can find information about treatment for a rare disease.

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Management Guidelines

  • The NORD Physician Guide  for Polycythemia vera was developed as a free service of the National Organization for Rare Disorders (NORD) and it's medical advisors.  The guides provide a resource for clinicians about specific rare disorders to facilitate diagnosis and treatment of their patients with this condition. 

Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Polycythemia vera. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
  • The Myeloproliferative Disorders Research Consortium (MPD-RC) is an international, multi-institutional non-profit consortium funded by the National Cancer Institute (NCI) at the National Institutes of Health and set up to coordinate, facilitate, and perform basic and clinic research investigating the genetics of MPDs with the goal of developing therapy. To learn more, click on the link.

Medical Products

The medication(s) listed in the table(s) below have been approved by the Food and Drug Administration (FDA) for treatment of this condition. The FDA Office of Orphan Products Development designates "orphan products" for those that treat rare diseases affecting fewer than 200,000 Americans. The table(s) below may not be an exhaustive list of drugs or products used to treat this condition. There may be other products available that are not considered orphan products. To search for all FDA approved drugs, visit Drugs@FDA. You can find orphan products used to treat other conditions by searching the Orphan Drug Product Designation database.


Generic Name Ruxolitinib Phosphate
Trade Name
(Manufacturer Name)
Jakafi
(Incyte Corporation)
Indication
The FDA has approved this product to be used in this manner.
Treatment of patients with intermediate or high-risk myelofibrosis, including primary myelofibrosis, post-polycythemia vera myelofibrosis and post-essential thrombocythemia myelofibrosis. Treatment of patients with polycythemia vera who have had an inadequate response to or are intolerant of hydroxyurea.
More Information about this product Drug Information Portal
Medline Plus Health Information

Other Names for this Disease
  • Acquired primary erythocytosis
  • Erythremia
  • Osler-Vaquez disease
  • Polycythemia rubra vera
  • Primary polycythemia
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.