March 1992

High- vs Low-Dose Immunoglobulin Therapy in the Long-term Treatment of X-linked Agammaglobulinemia

Author Affiliations

From the Division of Infectious Disease and Immunology, Department of Pediatrics, University of Munich (Drs Liese, Wintergerst, and Belohradsky) and the Department of Pediatrics, City Hospital, Harlaching, Munich (Dr Tympner) Federal Republic of Germany.

Am J Dis Child. 1992;146(3):335-339. doi:10.1001/archpedi.1992.02160150075025

• The data of 29 patients with X-linked agammaglobulinemia, who received immunoglobulin replacement therapy between 1965 and 1990, were analyzed for dose-dependent long-term results concerning infectious complications. Patients who received high-dose intravenous immunoglobulin replacement (>400 mg/kg every 3 weeks) showed a significant increase in trough serum IgG levels and a significant decrease in the incidence of pneumonias and the number of days spent in the hospital compared with patients receiving intravenous immunoglobulin low-dose (<200 mg/kg every 3 weeks) or intramuscular immunoglobulin (<100 mg/kg every 3 weeks) treatment. Improvements in therapeutic outcome were particularly evident when high-dose intravenous immunoglobulin replacement therapy was started before the age of 5 years. Bacterial meningitis, chronic pulmonary disease, and bronchiectasis occurred in the intramuscular immunoglobulin group but did not occur in either of the intravenous immunoglobulin groups. High-dose intravenous immunoglobulin therapy may have a positive impact on the clinical course and may prevent severe complications in patients with X-linked agammaglobulinemia.

(AJDC. 1992;146:335-339)