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