[Fulminant autoimmune cold-type hemolysis with marked elevation of monothermic cold agglutinins. Successful therapy with membrane plasmapheresis].
Autoimmune hemolytic anemia associated with cold autoantibodies is rare in infancy. In a 7 months old infant with severe hemolysis, a hemoglobin of 5.9 g/dl and a cold agglutinin titer of 1:8000, even the transfusion of warmed, packed red cells (37 degrees C) lead to hemolysis. Hemoglobin fell to 2.8 g/dl despite prevention of exposure to cold, parenteral steroids and immunoglobulins. Cold agglutinin titer fell to 1:8 after plasma separation. Subsequent transfusion did not lead to hemolysis and permanent remission was achieved.
Gordjani
N
N
Universitäts-Kinderklinik Marburg.
Kretschmer
V
V
Töllner
U
U
Rieger
C
C
D000373:Agglutinins / Q000032:analysis*; D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood* / Q000628:therapy; D003450:Cryoglobulins; D005260:Female; D006801:Humans; D007223:Infant; D010956:Plasmapheresis
D000373:Agglutinins; D003450:Cryoglobulins; C029443:cold agglutinins
Foudroyant verlaufende Autoimmunhämolyse vom Kältetyp mit ausgeprägter Vermehrung von monothermischen Kälteagglutininen: Erfolgreiche Therapie mit Membranplasmapherese.