Cold agglutinin disease
Other Names for this Disease
- Anemia, hemolytic, cold antibody
- Chronic cold agglutinin disease
- Cold agglutinin syndrome
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Your QuestionWhat is the difference between cryoglobulinemia and cold agglutinin disease?
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Cryoglobulinemia is a type of vasculitis that is caused by abnormal proteins (antibodies) in the blood called "cryoglobulins." At cold temperatures, these proteins become solid or gel-like, which can block blood vessels and cause a variety of health problems. Many people affected by this condition will not experience any unusual signs or symptoms. When present, symptoms vary but may include breathing problems; fatigue; glomerulonephritis; joint or muscle pain; purpura; Raynaud's phenomenon; skin death; and/or skin ulcers. In some cases, the exact underlying cause is unknown; however, cryoglobulinemia can be associated with a variety of conditions including certain types of infection; chronic inflammatory diseases (such as autoimmune disease); and/or cancers of the blood or immune system. Treatment varies based on the severity of the condition, the symptoms present in each person and the underlying cause.
Last updated: 5/7/2015
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). This eventually causes red blood cells to be prematurely destroyed (hemolysis) leading to anemia and other associated signs and symptoms. Cold agglutinin disease can be primary (unknown cause) or secondary, due to an underlying condition such as an infection, another autoimmune disease, or certain cancers. Treatment depends on many factors including the severity of the condition, the signs and symptoms present in each person, and the underlying cause.
Last updated: 5/6/2015
Although there is some overlap of symptoms, cryoglobulinemia and cold agglutinin disease differ in the process by which blood vessels become blocked. In cryoglobulinemia, antibodies accumulate and block blood vessels. In cold agglutinin disease, antibodies (different from those in cryoglobulinemia) attack and kill red blood cells, which then accumulate and block blood vessels.
Last updated: 5/7/2015
- Cryoglobulinemia. MedlinePlus. January 2013; http://www.nlm.nih.gov/medlineplus/ency/article/000540.htm.
- Adam M Tritsch, MD. Cryoglobulinemia. Medscape Reference. March 2015; http://emedicine.medscape.com/article/329255-overview.
- Salman Abdullah Aljubran, MD. Cold Agglutinin Disease. Medscape Reference. April 2015; http://emedicine.medscape.com/article/135327-overview.
- Anemia, Hemolytic, Cold Antibody. NORD. March 2008; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/723/viewFullReport.
- 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.