High- vs low-dose immunoglobulin therapy in the long-term treatment of X-linked agammaglobulinemia
J. G. Liese, U. Wintergerst, K. D. Tympner and B. H. Belohradsky
Department of Pediatrics, University of Munich, Federal Republic of Germany.
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
(greater than 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 (less than 200 mg/kg
every 3 weeks) or intramuscular immunoglobulin (less than 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.
