A Case of Severe Low-Titer Cold-Hemagglutinin Disease / 대한임상병리학회지

ABSTRACT

Most patients suffering from acute cold-hemagglutinin disease(CHAD) have cold-agglutinin titers well over 1000 which respond poorly to corticosteroids treatment. However, good results with corticosteroids have been reported in some patients with severe low-titer CHAD. We report the case of severe low-titer CHAD which responded well to corticosteroids treatment. A 7-year-old girl with unremarkable prior history was admitted in July 1999 with a three-day history of high fever and black-colored urine. Direct antiglobulin test using polyvalent and anti-C3d monovalent antisera were positive. Cold-agglutintin titer was as high as 1256 at 4degrees C, 132 at 25degrees C and less than 14 at 37degrees C. The serum contained cold autoantibodies against both adult and cord blood erythrocytes. Washed and prewarmed red blood cells were transfused. The girl experienced prompt relief of fever and hemolysis and improvement of her general condition after corticosteroids treatment.