Severe low-titer cold-hemagglutinin disease responsive to steroid pulse therapy

We report the case of a 12-year-old boy suffering from severe low-titer cold-hemagglutinin disease with excruciating colicky abdominal pain, jaundice, and acute hemolytic anemia requiring transfusion. Cold hemagglutinins of the IgM type and a positive direct antiglobulin test, predominantly against C3d, were found. Steroid pulse therapy with 20 mg/kg body wt. methylprednisolone for 3 consecutive days was given. Abdominal pain disappeared within 12 h of the first steroid infusion and hemolysis was halted. We conclude that a therapeutic trial with steroid pulse therapy in severe low-titer cold-hemagglutinin disease is warranted.