ABSTRACT

The first indication for the use of gammaglobulins was the treatment of a primary immuno-deficiency, Bruton’s or X-linked agamma-globulinemia. Since then, the efficacy in numerous open-label studies has been so clear that few clinicians would question its use in any of the severe primary humoral immuno-deficiencies. Most studies concluded that early intravenous immunoglobulin G (IgG) replacement therapy achieving residual IgG levels >5g/l is effective in preventing severe bacterial infections and pulmonary insufficiency. It could then be assumed that, concerning the treatment of primary immunodeficiencies, the story was over, but the presentations of the four posters in this session demonstrate that many problems still exist and require a better understanding. In particular, controversies exist regarding the need for a more intensive therapy required to prevent fully the onset of bronchiectasis, chronic sinusitis and non-bacterial infections, particularly enteroviral infections, in all cases, and the use of gamma globulin replacement in incomplete immunodeficiency states.