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Friday 18 May 2012

Paroxysmal cold hemoglobinuria

- rare form of AIHA occuring mostly in children
- usually triggered by a viral infection, usually self limited
- characterised by the involvement of Donath-Landsteiner antibody
- in vitro, this antibody has unique serologic feature: it has anti-P specificity and it binds to red cells only at low temperature (optimally at 4°C). When temperature is shifted to 37°C, lysis of red cells take place in the presence of complement
- consequently, in vivo there is intravascular hemolysis resulting in hemoglobinuria
- also presents with rigor and bone pain
- supportive treatment inlcuding blood transfusion is needed to control the anemia

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