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Abstract
The cold antibody autoimmune hemolytic anemias (AIHAs) are primarily comprised of
cold agglutinin syndrome (CAS) and paroxysmal cold hemoglobinuria (PCH) but, in addition,
there are unusual instances in which patients satisfy the serologic criteria of both
warm antibody AIHA and CAS ("mixed AIHA"). CAS characteristically occurs in middle-aged
or elderly persons, often with signs and symptoms exacerbated by cold. The responsible
antibody is of the IgM immunoglobulin class, is maximally reactive in the cold but
with reactivity up to at least 30 degrees C. Therapy is often ineffective, but newer
agents such as rituximab have been beneficial in some patients. PCH occurs primarily
in children, often after an upper respiratory infection. The causative antibody is
of the IgG immunoglobulin class and is a biphasic hemolysin that is demonstrated by
incubation in the cold followed by incubation at 37 degrees C in the presence of complement.
Acute attacks are frequently severe but the illness characteristically resolves spontaneously
within a few days to several weeks after onset and rarely recurs. Treatment consists
of supportive care, with transfusions frequently being needed.