[Clinical and immunologic aspects in patients with agammaglobulinemia]

Agammaglobulinemia is a rare disease. The concentration of these patients provides efficacy of diagnosis and treatment. Within this group the "acquired" type in adulthood (CVID, n = 31) can be separated from the X-linked agammaglobulinemia of infants (n = 6). The former is characterized by variable clinical and immunological pictures. Common to both are sinubronchial infections and extremely low levels of all classes of immunoglobulins. IgG-levels below 4 g/l are usually accompanied by a more severe disease. For the first time an osteopathy in most cases of CVID has been described. Patients with agammaglobulinemia should remain under close control with regard to their clinical data as well as to the result of the substitution by adequate preparations of immunoglobulins. A certain association with HLA-B21 could be identified. In 4 families with several afflicted members this has, however, not the case. Immunological screening in first-degree relatives provides information for genetical counseling.