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Pure Red Cell Aplasia, Acquired

Synonyms of Pure Red Cell Aplasia, Acquired

PRCA
Pure Red Blood Cell Aplasia

Disorder Subdivisions

No subdivisions found.

General Discussion

Acquired Pure Red Cell Aplasia is a rare bone marrow disorder characterized by an isolated decline of red blood cells (erythrocytes) produced by the bone marrow. Affected individuals may experience fatigue, lethargy, and/or abnormal paleness of the skin (pallor). Acquired Pure Red Cell Aplasia may occur for unknown reasons (idiopathic) or as a primary autoimmune disorder. It is also believed that Acquired Pure Red Cell Aplasia may occur secondary to a tumor of the thymus gland (thyoma), viral infections, or certain drugs.

Symptoms

Acquired Pure Red Cell Aplasia is characterized by a decrease in the number of red blood cells produced in the bone marrow. Individuals 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.

Affected individuals may experience fatigue, lethargy, and/or abnormal paleness of the skin (pallor).

Causes

Acquired 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 Populations

Acquired 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 Acquired 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.)

Standard Therapies

Acquired 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 affected individuals under 30 years of age, the disorder may initially be treated with the immune suppressant drug prednisone and/or antithymocyte globulin. The drugs cyclophosphamide, azathioprine, or 6-mercaptopurine which also suppress the immune system may be used for treating older individuals with Acquired Pure Red Cell Aplasia or those who fail to respond to steroids or antithymocyte globulin. Patients in both age groups may require periodic blood transfusions until the drugs take effect. The drug treatment is slowly decreased when remission of the disorder is acheived.

If an individual with Acquired Pure Red Cell Aplasia has a tumor of the thymus gland, surgical removal of this gland often causes remission of this disorder.

Investigational Therapies

Scientists are studying a number of drugs that suppress the immune system in people with Acquired Pure Red Cell Aplasia. More studies are needed to determine the long-term safety and effectivness of these treatments.

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Organizations related to Pure Red Cell Aplasia, Acquired

References

TEXTBOOKS
Hematology Basic Principles and Practice, 2nd Ed.: Ronald Hoffman M.D., et. al., Editors; Churchill-Livingstone, Inc., 1995. Pp. 350-70.

JOURNAL ARTICLES
Pure Red Cell Aplasia Characterized by Erythropoietic Maturation Arrest. Response to Anti-Thymocyte Globulin. A.D. Jacobs et al.; American Journal Med (Mar 1985; 78(3)). Pp. 515-17.

New Therapies for Aplastic Anemia. S.B. Krantz; American Journal Med Sciences (1986; 291). Pp. 371-79.

Diphenylhydantoin-Induced Pure Red Cell Aplasia. E.N. Dessypris et al.; Blood (1985; 65). Pp. 789-94.

Brief Report: Autoantibodies Against Erythropoietin in a Patient with Pure Red-Cell Aplasia. N. Casadevall et al.; New Eng J Med (Mar 7 1996; 334(10)). Pp. 630-33.

Successful Treatment of a Patient with a Thymoma and Pure Red-Cell Aplasia with Octreotide and Prednisone. G. Palmieri et al.; New Eng J Med (Jan 23 1997; 336(4)). Pp. 263-65.

Acquired Pure Red Cell Aplasia in Japan. S. Mamiya et al.; Eur J Haematol (Oct 1997; 59(4)). Pp. 199-205.

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Report last updated: 2007/08/07 00:00:00 GMT+0