Abstract
Autoimmune hemolytic anemia of childhood (AIHA) is often refractory to 2 mg/kg of prednisone. We report 2 infants with AIHA treated with initial doses of 5 gm/kg IVGG followed by maintenance doses of 2 gm/kg/wk. Infant #1 had required greater than 20 mg of prednisone/day for more than 6 months and had previously received 2 gm/kg of IVGG without apparent effect. After 5 gm/kg IVGG treatment his hemoglobin rose slowly over 4 weeks from 7.7 to 10.9, despite tapering of prednisone, and remains stable. Infant #2 had his second relapse of immune hemolytic anemia with the hemoglobin decreasing to 7 gm/dl. Two weeks of IVGG treatment stabilized his hemoglobin; 1½ weeks after treatment was stopped his hemoglobin fell to 4.5 gm/dl. Reinduction with 5 gm/kg IVGG and continued maintenance restabilized his hemoglobin at 7. Both patients had IgG antibodies which failed to bind to rH null cells after elution and abnormally decreased in vitro nonspecific antibody synthesis. Recent work by Salama et al demonstrates that a part of the RES Fc receptor blockade mediating an increased platelet count during IVGG treatment of ITP is due to low grade red cell immune hemolytic; therefore, they state that AIHA cannot be treated by IVGG. We demonstrate that AIHA can be treated by IVGG albeit at higher dosage than even in ITP (5 gm/kg vs 2 gm/kg). Other possible reasons for difficulty of treatment of AIHA by IVGG or steroids may include larger cell mass, lack of red cell Fc receptors or a more avid RES.
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Bussel, J., Cunningham-Rundles, C. & Hilgartner, M. INTRAVENOUS TREATMENT OF AUTOIMMUNE HEMOLYTIC ANEMIA WITH GAMMAGLOBULIN. Pediatr Res 18 (Suppl 4), 237 (1984). https://doi.org/10.1203/00006450-198404001-00862
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DOI: https://doi.org/10.1203/00006450-198404001-00862