Response to polysaccharide antigens in patients with ataxia-telangiectasia

Abstract

Fonte financiadora: FAPESP.Artigo submetido em 26.08.05, aceito em 09.11.05. AbstractObjective: To analyze the production of antibodies to polysaccharide antigens in patients with ataxia-telangiectasia. Patients and methods:We used the ELISA technique to measure the levels of IgG antibodies to serotypes 1, 3, 5, 6B, 9V and 14 of Streptococcus pneumoniae in 14 patients with ataxia-telangiectasia before and after immunization with 23-valent polysaccharide vaccine. Adequate response to individual polysaccharide can be defined as a postimmunization antibody titer equal to or greater than 1.3 µg/ml or as a minimum fourfold increase over the baseline (preimmunization) value.Results: Six (43%) patients showed an absent response to all serotypes analyzed. Four patients showed adequate response to only one serotype, one patient to two serotypes, two patients to three serotypes and only one patient to four out of six serotypes analyzed. No patient had adequate response to all serotypes tested. Postimmunization pneumococcus IgG levels were higher than preimmunization levels to all serotypes analyzed, except for serotype 3. In spite of this, the mean postimmunization levels were lower than 1.3 µg/ml in all serotypes, except for serotype 14. Mean increment was less than four in all serotypes analyzed. Conclusion:Our results suggest that patients with ataxiatelangiectasia are at a high risk of having an impaired response to pneumococcus, which may be one of the causes of recurrent sinopulmonary infections in these patients.J Pediatr (Rio J). 2006;82(2):132:6: Ataxia-telangiectasia, Streptococcus pneumoniae, pneumococcus, polysaccharide, immunodeficiency, humoral immunity, antibody. ResumoObjetivo: Estudar a produção de anticorpos a antígenos polissacarídicos em pacientes com ataxia-telangiectasia.Casuística e método: Utilizando a técnica de ELISA, determinamos os níveis de IgG aos sorotipos 1, 3, 5, 6B, 9V e 14 do pneumococo em 14 pacientes com ataxia-telangiectasia, antes e após a imunização com a vacina pneumocócica 23-valente. Resposta adequada a cada sorotipo foi definida como IgG > 1,3 µg/ml ou o incremento de quatro vezes dos níveis pós em relação aos pré-imunização.Resultados: Seis pacientes (43%) não responderam a todos os sorotipos analisados, quatro a apenas um sorotipo, um paciente a dois, dois a três e apenas um paciente a quatro dos seis sorotipos analisados. Nenhum paciente apresentou resposta adequada a todos os sorotipos testados. Os níveis de IgG ao pneumococo pós-imunização foram superiores aos pré-imunização para todos os sorotipos testados, exceto o 3. Apesar disso, os valores médios pós-imunização foram inferiores a 1,3 µg/ml para todos os sorotipos analisados, exceto o 14. A média de incremento da resposta foi inferior a quatro para todos os sorotipos analisados.Conclusão: Nossos resultados sugerem que pacientes com ataxiatelangiectasia têm grande risco de apresentar resposta inadequada ao pneumococo, o que pode ser uma das causas das infecções sinopulmonares de repetição.J Pediatr (Rio J). 2006;82(2):1...

Ataxia‐telangiectasia: recommendations for multidisciplinary treatment

“…76 Immunizations Most patients will have already received live viral vaccines (like measles, mumps, rubella [MMR] or varicella-zoster virus [VZV] vaccines) without complications before being diagnosed with ataxia-telangiectasia. [85][86][87] Expert guidelines recommend vaccination of all patients with ataxia-telangiectasia with 13-valent pneumococcal conjugate vaccine followed by 23-valent pneumococcal polysaccharide vaccine, repeated every 5 years. 76 Live oral polio vaccine should not be administered.…”

Evaluation and management of pulmonary disease in ataxia‐telangiectasia

“…People with A-T often have a suboptimal response to the 23-valent pneumococcal polysaccharide vaccine (34), as well as to other vaccines. Limited data suggest that the response to the 7-valent pneumococcal conjugate vaccine may be more robust (74).…”

Use and interpretation of diagnostic vaccination in primary immunodeficiency: A working group report of the Basic and Clinical Immunology Interest Section of the American Academy of Allergy, Asthma & Immunology

“…33,34 In both patients with Wiskott-Aldrich syndrome (WAS) and those with ataxia telangiectasia (AT), several small studies have reported poor responses to polysaccharide vaccines in the majority of patients. 35,36 A small study in patients with AT demonstrated that initial vaccination with PCV and subsequent vaccination with PPV could lead to higher antibody levels to PCV-specific serotypes and non-PCV serotypes. 37 The titers were determined by using ELISA with a PPV mix as the antigen.…”