In 1832 Elliotson described a patient who had cold "fits" and passed bloody urine "whenever the east wind blew." Dressler in 1854 described a patient with congenital syphilis who had intermittent attacks of albuminuria and red urine. He recognized that the color of urine was due to free hemoglobin and not to red cells. These two early reports illustrate the main features of paroxysmal cold hemoglobinuria—the occurrence of hemoglobinuria after chilling and the association of the disorder with syphilis.1 However, it was not until 1904 that the pathogenesis of the condition was delineated by Donath and Landsteiner.2 They demonstrated that the hemoglobinuria was caused by intravascular hemolysis induced by an autohemolysin in the patient's serum which combined with the red cell when the blood was chilled and produced hemolysis upon rewarming.
The following case of syphilitic paroxysmal cold hemoglobinuria is of particular interest with respect to the severity
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