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    Paroxysmal Cold Hemoglobinuria

    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

    Disorder Subdivisions

    • None

    General Discussion

    Summary
    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 sometimes 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 Diagnosis section ), especially in children with acute AIHA with hemoglobinuria.

    Introduction
    PCH was first described as a distinct disorder in the medical literature in 1872. The specific antibody associated with the disorder (Donath-Landsteiner autoantibody) was first described by Drs. Donath and Landsteiner in 1904.

    A majority of cases of 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. As children with PCH do not usually have hemolysis directly related to exposure to the cold (e.g., they continue hemolysis when in a warm hospital environment), it has been suggested that a better term might be Donath-Landsteiner test positive hemolytic anemia.

    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/

    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 (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: 6/7/2012
    Copyright 1990, 2003, 2008, 2012 National Organization for Rare Disorders, Inc.

    WebMD Medical Reference from the National Organization for Rare Disorders

    Last Updated: May 28, 2015
    This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

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