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.
Nanan
R
R
Children's Hospital, University of Würzburg, Germany.
Scheurlen
W
W
Gerlich
M
M
Huppertz
H I
HI
D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D001323:Autoantibodies / Q000097:blood; D002648:Child; D015933:Complement C3d / Q000276:immunology; D003298:Coombs Test; D006801:Humans; D007075:Immunoglobulin M / Q000276:immunology; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage / Q000627:therapeutic use*
D001323:Autoantibodies; D007075:Immunoglobulin M; D015933:Complement C3d; D008775:Methylprednisolone