Primary antibody deficiencies are characterized by decreased serum levels of immunoglobulin isotypes and increased susceptibility to infection by various microorganisms including encapsulated bacteria. This study was performed in order to evaluate bactericidal antibody response of these patients to polysaccharide meningococcal vaccine. Twenty-four antibody deficient children of mean age 11.2+/-3.5 years, and 15 sex and age-matched healthy volunteers were enrolled. All subjects received meningococcal polysaccharide vaccine A+C; blood samples were collected before vaccination and 3 weeks after vaccination. Following vaccination, the serum bactericidal antibody (SBA) geometric mean titre was significantly increased compared to the prevaccination level in the patient group (8.98 versus 1.63, P<0.001) and the control group (12.13 versus 1.26, P<0.001). All controls had a protective SBA response (SBA titre of >or=8 post-vaccination or rise of >or=4-fold from pre- to post-vaccination), whereas only 16 of 24 patients (66.6%) had a protective response (P=0.014). The non-responder patients included 5 cases with common variable immunodeficiency, two cases with hyper IgM syndrome, and one case with IgG subclass deficiency. This study indicates that some patients with primary antibody deficiencies can produce protective post-vaccination titres similar to the normal population, despite the common perception that patients with primary antibody deficiencies respond poorly to vaccination. This supports the use of polysaccharide-containing vaccines in these patients.