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Cold agglutinin disease
Cold agglutinin disease is an acquired autoimmune hemolytic anemia
due to an IgM autoantibody usually directed against the I antigen on red
blood cells. These IgM autoantibodies characteristically will not react
with cells at 37 degrees centigrade but only at lower temperatures.
In the cooler parts of the body (fingers, nose, ears), agglutination
of red blood cells by the IgM antibodies will transiently occur. Hemolysis
results indirectly from attachment of IgM, which in the cooler parts of
the circulation binds and fixes complement. When the red blood cell returns
to a cooler temperature, the IgM antibody dissociates, leaving complement
on the cell. Lysis, or destruction, of cells rarely occurs. Rather, C3b
present on the red cells is recognized by Kupffer cells (which have receptors
for C3b).
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Clinical suspicion
Extravascular hemolysis
Diagnosis
Extravascular hemolysis, Coomb test is positive for C3.
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