Paroxysmal Cold Hemoglobinuria

National Organization for Rare Disorders, Inc.

Important
It is possible that the main title of the report Paroxysmal Cold Hemoglobinuria 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

  • Donath-Landsteiner Hemolytic Anemia
  • Donath-Landsteiner Syndrome
  • Immune Hemolytic Anemia, Paroxysmal Cold
  • PCH

Disorder Subdivisions

  • None

General Discussion

A majority of cases of paroxysmal cold hemoglobinuria (PCH) recorded in the early medical literature were associated with late syphilis or congenital syphilis. In the early 1900s over 90 percent of patients with chronic PCH had a positive test for syphilis and approximately 30 percent showed clinical evidence of the disease. With the effective treatment of syphilis and the virtual elimination of the congenital form, "classical" syphilitic PCH is now an extremely rare disorder, as is chronic PCH. It was in patients with the chronic form of PCH that exposure to cold resulted in a paroxysm of hemoglobinuria. In modern times, PCH is almost always encountered as an acute transient syndrome in young children with a recent history of a viral illness, so that paroxysms resulting from cold exposure are rarely encountered. Thus, although this type of AIHA is known as PCH, the words paroxysmal and cold are generally not relevant to the disorder as it is manifest in the modern era.

Paroxysmal cold hemoglobinuria (PCH) is a rare type of anemia characterized by the premature destruction of healthy red blood cells by autoantibodies. The disorder is classified as an autoimmune hemolytic anemia (AIHA), an uncommon group of disorders in which the immune system mistakenly attacks healthy red blood cells. Autoimmune diseases occur when the body's natural defenses against foreign organisms destroy healthy tissue for unknown reasons. Normally, red blood cells have a life span of approximately 120 days before they get removed by the spleen. In individuals with PCH, red blood cells are destroyed prematurely and suddenly (paroxysmally). Many reports emphasize that PCH is an unusual disease. However, in recent years, PCH has become recognized as one of the most common causes of acute AIHA in young children. The reason why acute transient PCH appears to be a more common type of childhood AIHA than it was thought to be several decades ago is uncertain, but probably relates to greater awareness of the disorder and more frequent use of the Donath-Landsteiner test (see below), especially in children with acute AIHA with hemoglobinuria.

Resources

American Autoimmune Related Diseases Association, Inc.
22100 Gratiot Avenue
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
31 Center Drive MSC 2480
Building 31A Rm 4A16
Bethesda, MD 20892-2480
Tel: (301)592-8573
Fax: (240)629-3246
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
Email: ordr@od.nih.gov
Internet: http://rarediseases.info.nih.gov/Default.aspx

Anemia Institute for Research and Education
151 Bloor Street West
Suite 600
Toronto, Ontario, M5S 1S4
Canada
Tel: 416-969-7431
Fax: 416-969-7420
Tel: 877-992-6364
Email: info@anemiainstitute.org
Internet: http://www.anemiainstitute.org

AutoImmunity Community
Tel: (919) 552-9057
Email: bandrews@autoimmunitycommunity.org
Internet: http://autoimmunitycommunity.org

For a Complete Report

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). 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

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