Principles of and advances in immunoglobulin replacement therapy for primary immunodeficiency

Abstract

During the last 2 decades, the continued development and the large-scale production of polyclonal immune serum globulin (ISG) preparations with improved safety and tolerability profiles have allowed treatment to focus on quality of life and long-term freedom from the complications of primary immune deficiency disease, rather than just on freedom from severe acute infections and survival. Available ISG preparations allow routine therapy by a variety of routes and regimens that can be tailored to suit individual patients. Continued vigilance is required, however, because problems with emerging diseases, and the costs and availability of ISG are likely to present continuing challenges.

Fig. 1

Serum IgG concentrations in a 34-year-old man who has X-linked agammaglobulinemia. (Left panel) The diamonds and blue line indicate serum IgG concentrations at various times after a single intravenous infusion of 30 g (406 mg/kg). The black line indicates the average daily IgG level calculated by interpolation from points indicated by diamonds. Mean ± SEM = 850.6 ± 43 mg/dL. (Right panel) Serum IgG concentrations in the same patient receiving 12 g 16% IgG every 7 days. Mean ± SEM = 816 ± 16 mg/dL. (From Berger M. Subcutaneous immunoglobulin replacement in primary immunodeficiencies. Clin Immunol 2004;112:1–7; with permission.)