Paroxysmal Cold Hemoglobinuria: Report of a Case With an Exceptionally High Thermal Range Donath-Landsteiner Antibody

A patient with idiopathic paroxysmal cold hemoglobinuria had severe hemolytic anemia despite minimal exposure to cold. The patient’s autoantibody demonstrated all the characteristic features of Donath-Landsteiner antibodies, except that strong hemolysis occurred with simple cooling (monophasic hemolysis), as well as with traditional cold-warm incubation (biphasic hemolysis). Monophasic hemolysis was caused by a Donath-Landsteiner antibody of exceptionally high thermal range, which caused red cell sensitization and hemolysis up to 32°C. The patient’s severe hemolytic anemia was probably a result of the high thermal range of the autoantibody, which would allow intravascular hemolysis with even minimal exposure to cold. Corticosteroids had a beneficial effect in controlling hemolysis; however, strict environmental control, with vigorous prophylaxis against even minimal cold, was the most important part of the therapeutic program.