Pathogenesis and Clinical Features of Cold Agglutinin Disease
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Pathogenesis and Clinical Features of Cold Agglutinin Disease
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- Cold Agglutinin Disease (CAD, a type of Autoimmune Hemolytic Anemia, AIHA) is a rare disease, challenging at presentation and can present acutely or with more chronic symptoms
- CAD is a subtype of AIHA (accounting for ~20% of all AIHA) caused by IgM autoantibodies, which tend to react at cold temperatures; Cold Agglutinin Syndrome (CAS) is also an AIHA mediated by IgM, associated with systemic disease, most commonly infection or malignancy
- Hemolysis in CAD is complement-dependent with mainly extravascular hemolysis, typically in the liver
- CAD affects ~one person per million every year; middle-aged and elderly people, 40–80 years of age with average age of onset 60 years of age; more common in women than men
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