Paroxysmal cold haemoglobinuria. The most frequent acute autoimmune haemolytic anaemia in children?
Paroxysmal cold haemoglobinuria (PCH) is a disease which today is met mainly in its acute form in children. The diagnosis is revealed by a careful serological examination. Case reports of four children with PCH are given in this paper. They all showed typical clinical pictures of PCH, and displayed the expected serological findings, including a bithermic autoantibody (haemolysin), the Donath-Landsteiner antibody. Children with PCH often require immediate transfusion therapy, but certain precautions should be taken regarding transfusion policy in these patients. When the acute phase is overcome, the prognosis is excellent.
D000744:Anemia, Hemolytic, Autoimmune / Q000276:immunology* / Q000628:therapy; D001323:Autoantibodies / Q000032:analysis*; D001803:Blood Transfusion; D002675:Child, Preschool; D003080:Cold Temperature; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000276:immunology* / Q000628:therapy; D006460:Hemolysin Proteins / Q000032:analysis; D006461:Hemolysis; D006801:Humans; D008297:Male; D011379:Prognosis; D013577:Syndrome
D001323:Autoantibodies; D006460:Hemolysin Proteins