Antibody deficiency syndrome: a case with normal immunoglobulin levels.

Abstract

A girl is reported in whom abnormal susceptibility to infections in many sites, involving many types of bacteria and leading to fatal bronchopneumonia, was associated with normal levels of all immunoglobulins, but complete absence of isohaemagglutinins, and failure of antibody response to some bacterial antigens (Salmonella typhi O, Salmonella paratyphi B, O and H, and pertussis), possible delay in developing antibodies to streptococcal infection, but normal antibody production to other Salmonellae (O and H), staphylococci, diphtheria and tetanus toxoid. Histology of lymphoid tissues, and delayed type hypersensitivity and in vitro lymphocyte transformation were normal. This suggestive evidence of partial antibody deficiency syndrome, with normal concentration of all immunoglobulins, is compared with Aldrich's syndrome, and discussed as a possible instance of partial defect of the final stages of antibody synthesis or release.