The Turkish Journal of Pediatrics 2004 , Vol 46 , Num 3
Defective anti-polysaccharide antibody response in patients with ataxia-telangiectasia

Departments of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey

Departments of Molecular Biology, Hacettepe University Faculty of Medicine, Ankara, Turkey

Departments of Pathology, UCLA School of Medicine, Los Angeles CA, USA

Abstract

Sanal Ö, Özbaş-Gerçeker F, Yel L, Ersoy F, Tezcan I, Berkel Ay, Metin A, Gatti RA. Defective anti-polysaccharide antibody response in patients with ataxia-telangiectasia. Turk J Pediatr 2004; 46: 208-213.

The immunodeficiency in ataxia-telangiectasia (A-T) patients involves both cellular and humoral immunity; however, the specific antibody response is not well defined. Frequent respiratory infections are a prominent feature in A-T. Streptococcus pneumoniae is a common pathogen responsible for these infections. Defective B cell membrane signaling has been reported in A-T cells. These observations prompted us to investigate the B cell response to six frequently encountered pneumococcal serotypes in A-T patients. We found defective IgG antibody production to all studied serotypes (3, 6B, 7F, 14, 19F, and 23F) in 22 of 31 A-T patients (71%) who were immunized with a polyvalent pneumococcal vaccine. The impaired antibody responses did not correlate with either history of infection or serum immunoglobulin isotype levels. In addition, we did not observe any correlation between the pneumococcal antibody production and a specific mutation or level of intracellular ATM (ataxiatelangiectasia mutated) protein in lysates of lymphoblastoid cell lines from these patients. Our results suggest that the extent and severity of the recurrent sinopulmonary infections may depend not only on the immunological defects but also on other ATM-dependent physiological responses.

Keywords : ataxia-telangiectasia pneumococcal vaccine antibody response Atm mutation.
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