Abstract
IVIG has been used to treat AIN, but it is not always effective. We report a patient in which the concommitant use of IVIG and steroid was more effective than either alone. A 10 year old girl with neutropenia and anti-neutrophil antibodies had chronic gingivitis, buccal erosions, and staphyloccal skin abscesses. Absolute neutrophil counts (ANC) were 40-400/mm3. IVIG at 2 gm/kg had no effect. She later developed fever, anorexia, and abdominal pain due to bacterial ileitis. Antibiotics alone gave only partial clinical improvement. The addition of Solumedrol, 1 mg/kg/day, transiently raised the ANC from 100 to 3500. The ANC subsequently declined to <100, with clinical relapse, while on the same steroid dose. Adding 1 gm/kg/day IVIG X 3 raised the ANC to 2400 in 2 days and to 11000 in 6 days. This allowed resection of the infected ileum without complications. The ANC remained >1000 for 30 days. Subsequently, while on 40/20 mg alternate day prednisone, 4 infusions of 1 gm/kg IVIG over 7 days did not raise the ANC. Increasing the steroids to 45 mg/day (1 mg/kg) promptly increased the ANC from 800 to 9700 without further IVIG. Ten monthly 1 gm/kg infusions of IVIG have kept her infection-free by maintaining ANC >500, although anti-neutrophil antibodies continue to be present. As prednisone was tapered and discontinued, the ANC response to each course of IVIG decreased. Optimization of prednisone dose in relation to the IVIG infusions is in progress. Our observations suggest that IVIG and steroids may act synergistically to decrease peripheral destruction of antibody sensitized blood cells.
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Kallick, M., Sorensen, R. & Berger, M. SYNERGISTIC EFFECT OF STEROIDS AND INTRAVENOUS IMMUNE GLOBULIN (IVIG) IN A PATIENT WITH AUTOIMMUNE NEUTRO PENIA (AIN). Pediatr Res 21 (Suppl 4), 313 (1987). https://doi.org/10.1203/00006450-198704010-00873
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DOI: https://doi.org/10.1203/00006450-198704010-00873