Cold agglutinin disease (CAD) is a rare autoimmune disease characterized by the presence of high concentrations of circulating cold sensitive antibodies, usually IgM and autoantibodies that are also active at temperatures below 30 °C (86 °F),[1] directed against red blood cells, causing them to agglutinate and undergo lysis.[2] It is a form of autoimmune hemolytic anemia, specifically one in which antibodies bind red blood cells only at low body temperatures, typically 28–31 °C.
When affected people's blood is exposed to cold temperatures (32 °F (0 °C; 273 K) to 50 °F (10 °C; 283 K)), 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.[3][4]
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.[3][4]
Cold agglutinin disease was first described in 1957.[5][6]
^"Cold agglutinin disease". Orphanet. Archived from the original on 2015-10-06. Retrieved 2019-02-09.
^Cold Agglutinin Disease at eMedicine
^ ab"Anemia, Hemolytic, Cold Antibody". NORD (National Organization for Rare Disorders). 2004-10-28. Archived from the original on 2017-02-21. Retrieved 2019-02-09.
^ abCite error: The named reference Genetic and Rare Diseases Information Center (GARD) – an NCATS Program 2019 was invoked but never defined (see the help page).
^Dacie, J. V.; Crookston, J. H.; Christenson, W. N. (1957). "'Incomplete' Cold Antibodies: Role of Complement in Sensitization to Antiglobulin Serum by Potentially Haemolytic Antibodies". British Journal of Haematology. 3 (1): 77–87. doi:10.1111/j.1365-2141.1957.tb05773.x. PMID 13413095. S2CID 38159450.
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