Evans Syndrome

National Organization for Rare Disorders, Inc.

Important
It is possible that the main title of the report Evans Syndrome is not the name you expected.

Disorder Subdivisions

  • None

General Discussion

Summary
Evans syndrome is a rare disorder in which the body's immune system produces antibodies that mistakenly destroy red blood cells, platelets and sometimes certain white blood cell known as neutrophils. This leads to abnormally low levels of these blood cells in the body (cytopenia). The premature destruction of red blood cells (hemolysis) is known as autoimmune hemolytic anemia or AIHA. Thrombocytopenia refers to low levels of platelets (idiopathic thrombocytopenia purpura or ITP in this instance). Neutropenia refers to low levels of certain white blood cells known as neutrophils. Evans syndrome is defined as the association of AIHA along with ITP; neutropenia occurs less often. In some cases, autoimmune destruction of these blood cells occurs at the same time (simultaneously); in most cases, one condition develops first before another condition develops later on (sequentially). The symptoms and severity of Evans syndrome can vary greatly from one person to another. Evans syndrome can potentially cause severe, life-threatening complications. Evans syndrome may occur by itself as a primary (idiopathic) disorder or in association with other autoimmune disorders or lymphoproliferative disorders as a secondary disorder. (Lymphoproliferative disorders are characterized by the overproduction of white blood cells.) The distinction between primary and secondary Evans syndrome is important as it can influence treatment.

Introduction
Evans syndrome was first described in the medical literature in 1951 by Dr. Robert Evans and associates. For years, the disorder was considered a coincidental occurrence of AIHA with thrombocytopenia and/or neutropenia. However, researchers now believe that the disorder represents a distinct condition characterized by a chronic, profound (more than in ITP or AIHA alone) state of immune system malfunction (dysregulation).

Resources

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/

Autoimmune Information Network, Inc.
PO Box 4121
Brick, NJ 08723
Fax: (732)543-7285
Email: autoimmunehelp@aol.com

AutoImmunity Community
Email: moderator@autoimmunitycommunity.org
Internet: http://www.autoimmunitycommunity.org

Evans Syndrome Community Network
400 18th Place
West Des Moines, IA 50265
Tel: (515)276-1836
Fax: (515)276-1836
Email: ESCN@me.com
Internet: http://www.evanssyndrome.net

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. 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.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  10/25/2013
Copyright  2013 National Organization for Rare Disorders, Inc.