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$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.