Pure Red Cell Aplasia, Acquired

Pure Red Cell Aplasia, Acquired

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Pure Red Cell Aplasia, Acquired is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • PRCA
  • Pure Red Blood Cell Aplasia

Disorder Subdivisions

  • None

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.

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

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.

Resources

Aplastic Anemia & MDS International Foundation, Inc.

100 Park Avenue, Suite 108

Rockville, MD 20850

USA

Tel: (301)279-7202

Fax: (301)279-7205

Tel: (800)747-2820

Email: help@aamds.org

Internet: http://www.aamds.org



American Autoimmune Related Diseases Association, Inc.

22100 Gratiot Ave.

Eastpointe, MI 48021

Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

Internet: http://www.aarda.org/



NIH/National Heart, Lung and Blood Institute

P.O. Box 30105

Bethesda, MD 20892-0105

Tel: (301)592-8573

Fax: (301)251-1223

Email: nhlbiinfo@rover.nhlbi.nih.gov

Internet: http://www.nhlbi.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



AutoImmunity Community

Email: moderator@autoimmunitycommunity.org

Internet: http://www.autoimmunitycommunity.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

This information does not replace the advice of a doctor. Healthwise, Incorporated disclaims any warranty or liability for your use of this information. Your use of this information means that you agree to the Terms of Use . How this information was developed to help you make better health decisions.

Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.