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What is Cold agglutinin disease

Cold agglutinin disease is a form of autoimmune hemolytic anemia caused by cold-reacting autoantibodies (a type of protein produced by the immune system). Primary cold agglutinin disease is usually associated with monoclonal (produced from a single ancestral cell by repeated cellular replication) cold-reacting autoantibodies. Primary cold agglutinin disease is chronic and occurs after the fifth decade of life, with a peak incidence in the seventh and eighth decades. Secondary cold agglutinin disease is predominantly caused by infection and lymphoproliferative disorders in which lymphocytes (white blood cells) are produced in excessive quantities. It is essential with chronic cold agglutinin disease to keep all body parts warm at all times and avoid cooling of body parts. Appropriate clothing is necessary for cold environments, and avoidance of cold foods and working in cold storage areas is also important.

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.[1][2][3]

Symptoms of cold agglutinin disease (CAD) are often triggered or made worse by cold temperatures or a viral infection. Therefore, symptoms generally are worse during winter months. Symptoms may arise suddenly or gradually.[4]

This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
https://rarediseases.info.nih.gov/diseases/6130/cold-agglutinin-disease

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