$Unique_ID{BRK04120} $Pretitle{} $Title{Polycythemia Vera} $Subject{Polycythemia Vera Primary Polycythemia Vaquez-Osler Disease Osler-Vaquez Disease Splenomegalic Polycythemia Erythremia} $Volume{} $Log{} Copyright (C) 1986, 1990 National Organization for Rare Disorders, Inc. 236: Polycythemia Vera ** IMPORTANT ** It is possible the main title of the article (Polycythemia Vera) is not the name you expected. Please check the SYNONYMS listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Primary Polycythemia Vaquez-Osler Disease Osler-Vaquez Disease Splenomegalic Polycythemia Erythremia General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Polycythemia Vera is a chronic proliferative disorder of the bone marrow. It is characterized by an increase in the number of red blood cells (erythrocytosis) and hemoglobin concentration in the blood. Symptoms Initial symptoms of Polycythemia Vera are fatigue, malaise, difficulty concentrating, headache, drowsiness, forgetfulness, and dizziness. Itchiness is present in 50% of patients, particularly after a hot bath. Redness of the skin may be seen, or patients may have normal skin color with only dusky redness of the mucous membranes. The retinal veins of the eyes may be dark red, full and tortuous. The spleen is usually palpable. Some patients do not have any obvious symptoms when Polycythemia is diagnosed. Causes The cause of Polycythemia Vera is not known. Related Disorders Leukemia is a generalized cancerous (neoplastic) disorder of the blood forming tissues, primarily of the white blood cell (leukocytic) type. Therapies: Standard The most common therapy for Polycythemia vera is removal of some blood from a vein (phlebotomy). This does not depress marrow function nor does it induce genetic mutation. Iron-deficiency anemia usually develops following repeated phlebotomy. Phlebotomy is less effective in patients with high iron absorption rates or greatly elevated platelet counts, and is inconvenient in patients with poor veins. Usually 3 to 6 phlebotomies are required to reduce the percentage of red blood cell volume in the blood (Hct) to less than 50%, returning the red blood cell number to normal. Elderly patients with advanced arteriosclerosis or a heart condition should have less blood removed at each phlebotomy. When phlebotomy is the only therapy, supplemental iron must not be prescribed since it tends to accelerate hemoglobin production. A low-iron diet is often impractical, but foods with very high iron content, such as clams, oysters, liver and legumes, should be avoided. Phlebotomy followed radiophosphorus (32P) therapy, produces clinical and hematologic remission in almost all patients. Remissions usually last 18 months, but vary from 6 months to several years. Fewer follow-up visits are often required and there are usually no immediate side effects. However, approximately 10% of patients treated with 32P develop acute leukemia, but this usually occurs after more than 10 years of treatment. Chemotherapy for Polycythemia Vera is no longer advised because it increases the risk of developing acute leukemia. An excess of uric acid in the blood (hyperuricemia) requires treatment with the drug allopurinol. Acute gouty arthritis can develop and it is treated in the same manner as primary gout; i.e., with colchicine or nonsteroidal anti-inflammatory drugs for patients who are intolerant of colchicine. Corticosteroids can produce rapid and complete remission in some patients, but generally are used only when other drugs are ineffective. Therapies: Investigational The FDA allows the use of the orphan drug Anagrelide for Polycythemia Vera on an experimental or investigational basis. This drug is manufactured by Roberts Pharmaceutical Corporation, Meridian Center III, 6 Industrial Way West, Eatontown, NH, 07724, (908) 389-1182. This disease entry is based upon medical information available through June 1989. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Polycythemia Vera, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Myeloproliferative Research Center, Inc. 2220 Tiemann Ave. Baychester, NY 10469 (718) 231-0270 (800) MPD-HELP National Heart, Lung and Blood Institute 9000 Rockville Pike Bethesda, MD 20892 (301) 496-4236 References THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme Research Laboratories, 1987. P. 1138. CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Eds.: W. B. Saunders Co., 1988. Pp. 973, 980-4.