Anti-peptide Antibody Responses in Patients with Ataxia-telangiectasia
Mahnaz Jamee1, 2, Laleh Sharifi3 , Saleh Ghiasy
Corresponding author: Saleh Ghiasy E-mail: saleh.ghiasy@yahoo.com 1. Student Research Committee, Alborz University of Medical Sciences, Alborz, Iran 2. Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran 3. Uro-Oncology Research Center, Tehran University of Medical Sciences, Tehran, Iran 4. Cancer Research Center, Sha
Article 3, Volume 2, Issue 1, Winter 2019, Page 28-36 XML PDF (808.47 K) Document Type: Original Article DOI: 10.22034/IGJ.2019.85745 Abstract Background/Objectives: Ataxia-telangiectasia (AT) is a rare inherited disorder caused by mutations in the ATM (Ataxia Telangiectasia Mutated) gene. Antibody response to diphtheria and tetanus toxoid vaccines may reveal indirect information about both cellular and humoral arms of the immune system in these patients. This study, therefore, set out to assess the specific antibody responses against tetanus and diphtheria vaccination among AT patients. Methods: Thirty-eight AT patients were entered the study and an appropriate questionnaire was completed for all of them. Laboratory findings including alpha fetoprotein, lymphocyte subsets, serum immunoglobulin levels of IgG, IgG subsets, IgA, IgM, IgE and antibody response against diphtheria and tetanus toxoids were measured. Results: Thirty-eight A-T patients were enrolled in this study. Based on the anti-tetanus and anti-diphtheria antibody production, 24 and 14 patients were categorized in responder (R) and non-responder (NR) groups, respectively. Respiratory tract infection was the most common infectious complication reported more frequently in the R comparing to NR group. Within the non-infectious manifestations, after cerebellar ataxia, ocular telangiectasia (52.6%) and FTT (26.3%) were the most frequent. 34.8% of individuals in R group but none of the NR patients had normal serum immunoglobulin profile (P=0.015). Contrarily, HIGM phenotype was found more frequent in NR group comparing to R group (50% vs. 17.4%, p= 0.063). Conclusions: In accordance with the previous studies, we observed sufficient antibody response to diphtheria and tetanus vaccines in most of the AT patients. Keywords Ataxia telangiectasia; immune deficiency; specific antibody response; anti-peptide antibody; polypeptide vaccine; humoral immune defect
Doi: 10.22034/igj.2019.166485.1008