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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Cold agglutinin disease


Other Names for this Disease
  • Anemia, hemolytic, cold antibody
  • CAD
  • CAS
  • Chronic cold agglutinin disease
  • Cold agglutinin syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Symptoms

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What are the signs and symptoms of Cold agglutinin disease?

Cold agglutinin disease is a rare type of autoimmune hemolytic anemia in which the body's immune system mistakenly attacks and destroys its own red blood cells. When affected people's blood is exposed to cold temperatures (32º to 50º F), certain proteins that normally attack bacteria (IgM antibodies) attach themselves to red blood cells and bind them together into clumps (agglutination). The antibodies then activate other components of the blood, which eventually causes red blood cells to be prematurely destroyed. As the number or red blood cells drop, affected people typically experience anemia, which may be associated with pallor, weakness, fatigue, irritability, headaches, and/or dizziness.[1]

Other signs and symptoms of cold agglutinin disease vary, but may include:[2][1][3]
  • Painful fingers and toes with purplish discoloration
  • Abnormal behavior
  • Amenorrhea
  • Gastrointestinal issues
  • Dark urine
  • Enlargement of the spleen
  • Jaundice
  • Heart failure
  • Shock
Last updated: 5/5/2015

The Human Phenotype Ontology provides the following list of signs and symptoms for Cold agglutinin disease. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.

Signs and Symptoms Approximate number of patients (when available)
Arthralgia 90%
Autoimmunity 90%
Hemolytic anemia 90%
Muscle weakness 90%
Pallor 90%
Abnormality of urine homeostasis 7.5%
Diarrhea 7.5%
Hepatomegaly 7.5%
Lymphadenopathy 7.5%
Migraine 7.5%
Nausea and vomiting 7.5%
Splenomegaly 7.5%

Last updated: 7/1/2015

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.


References
  1. Anemia, Hemolytic, Cold Antibody. NORD. March 2008; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/723/viewFullReport.
  2. Salman Abdullah Aljubran, MD. Cold Agglutinin Disease. Medscape Reference. April 2015; http://emedicine.medscape.com/article/135327-overview.
  3. Stanley L Schrier, MD; Wendell F Rosse, MD. Clinical features and treatment of autoimmune hemolytic anemia: Cold agglutinins. UpToDate. December 2014; Accessed 5/5/2015.


Other Names for this Disease
  • Anemia, hemolytic, cold antibody
  • CAD
  • CAS
  • Chronic cold agglutinin disease
  • Cold agglutinin syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.