cold agglutinin syndrome

cold agglutinin syndrome
the presence of circulating cold agglutinins, usually IgM, which are directed against three types of polysaccharide red cell antigens: I antigens, expressed primarily on adult red cells, i antigens, expressed primarily on cells of fetuses and infants, and Pr antigens, which, unlike I and i antigens, are protease sensitive. The primary clinical manifestations are intravascular hemolysis in exposed extremities and mild hemolytic anemia due to complement fixation, both occurring only upon exposure to cold. There are two major types: chronic cold agglutinin disease, a condition seen in the elderly with gradual onset and a chronic course; and postinfectious cold agglutinin syndrome, which usually follows Mycoplasma pneumoniae infection or infectious mononucleosis and lasts a few months. The syndrome can also develop secondary to malignancy.

Medical dictionary. 2011.

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