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$Unique_ID{BRK04155}
$Pretitle{}
$Title{Pure Red Cell Aplasia}
$Subject{Pure Red Cell Aplasia Pure Red Blood Cell Aplasia PRCA Anemia,
Aplastic Blackfan-Diamond Anemia Fanconi's Anemia}
$Volume{}
$Log{}
Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.
506:
Pure Red Cell Aplasia
** IMPORTANT **
It is possible the main title of the article (Pure Red Cell Aplasia) is
not the name you expected. Please check the SYNONYMS listing on the next
page to find alternate names and disorder subdivisions covered by this
article.
Synonyms
Pure Red Blood Cell Aplasia
PRCA
Information on the following disorders may be found in the Related
Disorders section of this report:
Anemia, Aplastic
Blackfan-Diamond Anemia
Fanconi's Anemia
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.
Pure Red Cell Aplasia is a rare blood disorder characterized by a sudden
decrease in the number of red blood cells (erythrocytes) produced in the bone
marrow.
Symptoms
Pure Red Cell Aplasia is characterized by a sudden decrease in the number of
red blood cells produced in the bone marrow. Persons with this disorder are
deficient in the number of precursors of red blood cells (erythroblasts).
Levels of the hormone erythropoietin that stimulates the bone marrow to
produce red blood cells are usually elevated.
Causes
Pure Red Cell Aplasia is thought to be an autoimmune disorder possibly caused
either by a tumor of the thymus gland, certain drugs or a viral infection.
It is one of a group of bone marrow failure syndromes.
Affected Population
Pure Red Cell Aplasia is a rare disorder affecting males and females in equal
numbers.
Related Disorders
Symptoms of the following disorders are similar to those of Pure Red Cell
Aplasia. Comparisons may be useful for a differential diagnosis:
Aplastic Anemia is characterized by failure of the bone marrow to produce
red blood cells, white blood cells and platelets. Certain other anemias are
due either to excessive red cell destruction or a limited production of red
blood cells. Aplastic Anemia may occur for unknown reasons, or it may be the
result of a toxic reaction to radiation, certain drugs or chemicals. In rare
cases, the disorder may be caused by a tumor in the thymus gland. (For more
information on this disorder, choose "Aplastic Anemia" as your search term in
the Rare Disease Database.)
Blackfan-Diamond Anemia is a very rare genetic blood disorder which is
present at birth. Blood cell abnormalities accompany an unusual physical
appearance, paleness, weakness, and lethargy. (For more information on this
disorder, choose "Blackfan" as your search term in the Rare Disease
Database.)
Fanconi's Anemia is a rare form of familial aplastic anemia. It is
characterized by bone abnormalities, an abnormally small head (microcephaly),
decreased functioning of the sex organs (hypogenitalism) and brown
pigmentation of the skin. Complications may include infections such as
pneumonia, meningitis, excessive bleeding (hemorrhages), and leukemia. Other
malignancies may also occur. (For more information on this disorder, choose
"Fanconi" as your search term in the Rare Disease Database.)
Therapies: Standard
Pure Red Cell Aplasia usually goes into remission when certain drugs such as
sulfonylureas (used for treating diabetes), gold for treatment of arthritis,
penicillin, phenytoin and phenobarbitol used for treating epilepsy, or the
anesthetic halothane which can cause this disorder are discontinued. In
patients under 30 years of age, the disorder may initially be treated with
the anti-inflammatory drug prednisone and anti-thymocyte globulin. The drugs
cyclophosphamide, azathioprine, or 6-mercaptopurine which are toxic for
certain cells, and which suppress the immune system (cytotoxic
immunosuppressive), may be used for treating older patients with Pure Red
Cell Aplasia. After the immunosuppressive therapy begins, patients in both
age groups may require periodic blood transfusions until the drugs take
effect. The drug treatment is discontinued when remission of the disorder is
achieved.
For patients who remain resistant to conventional immunosuppressive
therapies, antihuman thymocyte gamma globulin may be used. This drug is
produced by immunizing horses or rabbits with human thymus cells, and
collecting the gamma globulin from their blood plasma.
If a patient with Pure Red Cell Aplasia has a tumor of the thymus gland,
surgical removal of this gland often causes remission of this disorder.
Therapies: Investigational
This disease entry is based upon medical information available through August
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 Pure Red Cell Aplasia, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
NIH/National Heart, Lung and Blood Institute (NHLBI)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-4236
Aplastic Anemia Foundation of America
P.O. Box 22689
Baltimore, MD 21203
(301) 955-2803
1-800-747-2820
References
PURE RED CELL APLASIA CHARACTERIZED BY ERYTHROPOIETIC MATURATION ARREST.
RESPONSE TO ANTI-THYMOCYTE GLOBULIN: A.D. Jacobs, et al.; American Journal
Med (March 1985: issue 78(3)). Pp. 515-517.
NEW THERAPIES FOR APLASTIC ANEMIA: S.B. Krantz; American Journal Med
Sciences (1986: issue 291). Pp. 371-379.
DIPHENYLHYDANTOIN-INDUCED PURE RED CELL APLASIA: E.N. Dessypris, et al.;
Blood (1985: issue 65). Pp. 789-794.