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Paroxysmal cold hemoglobinuria
The Donath-Landsteiner cold hemolysin is an unusual IgG antibody with
anti-P specificity that was originally noted in cases of congenital or
acquired syphilis. The disease it causes is termed "paroxysmal cold hemoglobinuria."
Hemolysis in this syndrome most commonly occurs intravascularly, after
the antibody has passed through a cell attachment phase in the lower temperatures
of the peripheral circulation. The intravascular hemolysis is due to the
unusual complement-activating efficiency of this IgG antibody. As its
name implies, this antibody is associated with cold hemoglobinuria. This
antibody, although uncommon, is most frequently found in children with
viral infections. Hemolysis, even though sometimes severe, is usually
mild and tends to resolve as the infection clears.

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Diagnosis
Intravascular hemolysis + direct Coomb test positive for
complement only and active at 4șC.
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