Autoimmune haemolysis associated with Donath-Landsteiner antibodies.
The clinical pattern of haemolysis associated with Donath-Landsteiner antibodies has undergone a change over the years. In the current study 13 patients developed the acute form of the disease whilst only 1 presented with the classical picture of chronic paroxysmal cold haemoglobinuria. The acute illness typically occurred in young children with a male predominance of about 2.5:1; 10 patients were less than 5 years old. There was often a history of a preceding infection, the onset was sudden, prostration, haemoglobinuria and pallor were prominent. The patients were very ill but rapid and complete recovery usually occurred within a few days; however, 1 patient died. Treatment consisted of rest and warmth; in addition blood transfusion was needed in 7 patients. The chronic disease (which was non-syphilitic in origin) followed a benign course, warmth and avoidance of cold being all that were necessary to maintain the patient's well-being. It is felt that the general term for this disorder should be Donath-Landsteiner haemolysis rather than the traditional paroxysmal cold haemoglobinuria.
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000276:immunology* / Q000628:therapy; D001323:Autoantibodies / Q000032:analysis*; D002675:Child, Preschool; D002908:Chronic Disease; D003080:Cold Temperature; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000276:immunology* / Q000628:therapy; D006460:Hemolysin Proteins / Q000276:immunology*; D006461:Hemolysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D012737:Sex Factors; D009626:Terminology as Topic
D001323:Autoantibodies; D006460:Hemolysin Proteins