Abstract
Paroxysmal cold haemoglobinuria (PCH) is an acquired cause of haemolytic anaemia. It is caused by the biphasic IgG antibodies that sensitize and attach the red cells at lower temperature; detach and rebind on rewarming. Donath-Landsteiner antibody test is the diagnostic test for PCH. Management of PCH mainly includes supportive care but sometimes disease becomes severe and life-threatening. We describe a similar severe and life-threatening case of PCH who was managed by plasma exchange followed by packed red cell transfusion.