Paroxysmal cold hemoglobinuria and cardiopulmonary bypass

Paroxysmal cold hemoglobinuria, a cold-reactive autoimmune disease associated with the Donath-Landsteiner antibody, has not been described in patients undergoing cardiac surgery. We report a case of mitral valve replacement in a woman with a positive Donath-Landsteiner antibody and a history of recurrent hemolysis and hemoglobinuria secondary to cold exposure. Successful perioperative management is described, as is a discussion of paroxysmal cold hemoglobinuria.