How is episodic hemoglobinuria following cold exposure differentiated from cold agglutinin disease?

Updated: Aug 28, 2018
  • Author: Salman Abdullah Aljubran, MD; Chief Editor: Michael A Kaliner, MD  more...
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Answer

This can occur in more severe cases of cold agglutinin disease, so confusion may exist as to whether a patient has Donath-Landsteiner hemolytic anemia or cold agglutinin disease. The cold agglutinin titer is less than 1:64 in Donath-Landsteiner, and the result of the Donath-Landsteiner test for cold hemolysis is positive. (Donath-Landsteiner screening tests are warranted only if hemoglobinuria is detected in the patient.) The age of the patient is very important, as a diagnosis of Donath-Landsteiner hemolytic anemia is more probable in a child; in one study, the median age at presentation was 5 years and the peak incidence was in children 4 years and under. [27] March hemoglobinuria (episodic) is clearly related to exertion.


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