B Cells and T Cells Abnormalities in Patients With Selective IgA Deficiency

BACKGROUND: Selective IgA deficiency (SIgAD) is the most prevalent inborn errors of immunity with almost unknown etiology. This study aimed to investigate the clinical diagnostic and prognostic values of lymphocyte subsets and function in symptomatic SIgAD patients. METHODS: A total of 30 available SIgAD patients from the Iranian registry and 30 age-sex-matched healthy controls were included in the present study. We analyzed B and T cell peripheral subsets and T cell proliferation assay by flow cytometry in SIgAD patients with mild and severe clinical phenotypes. RESULTS: Our results indicated a significant increase in naïve and transitional B cells and a strong decrease in marginal zone-like and switched memory B-cells in SIgAD patients. We found that naïve and central memory CD4 CONCLUSION: SIgAD patients have varied cellular and humoral deficiencies. Therefore, T cell and B cell assessment might help in better understanding the heterogeneous pathogenesis and prognosis estimation of the disease

Impact of Age on Survival of Patients with Operable Breast Cancer

Background: Breast cancer arising in young patients (? 40 years) is being considered as a distinct clinical entity with more aggressive tumor features and poorer survival. Our aim was to assess the impact of age on survival among a large group of Iranian women diagnosed with breast cancer. Methods: In a cross-sectional study, demographic and clinicopathological characteristics of patients with breast cancer who were treated in two referral centers in Tehran, Iran during the past 13 years were reviewed and extracted from an electronic database. Patients were divided into two groups based on the age at the time of diagnosis (?40 and >40 years). The association of age with different clinicopathological features and its impact on disease-free survival were assessed. Results: Study population comprised of 353(26.1%) patients who were 40 years old or younger and 1000(73.9%) who were older. Compared to older patients, younger participants had more commonly tumor size larger than 5 cm (P = 0.034), higher chance of lymph node metastasis (P = 0.036), and overexpression of HER-2 (P = 0.004). No significant differences were observed between the two groups regarding ER, PR, and LNR (lymph node ratio). Age was the only factor affecting patients' disease-free survival and younger patients had higher chance of local or distant metastases compared to older subjects (HR: 1.49, 95%CI: 1.02-2.17, P = 0.038). Conclusions: Based on the results of current study, it can be suggested that younger patients who are diagnosed with breast cancer tend to have larger tumor size, higher chance of lymph node metastasis and overexpression of HER-2 compared to patients older than 40 years. Age was the only significant factor that was associated with shorter disease-free survival

Cohort of Iranian Patients with Congenital Agammaglobulinemia: Mutation Analysis and Novel Gene Defects

<p><b>Objectives:</b> Impairment in early B-cell development can cause a predominantly antibody deficiency with severe depletion of peripheral B-cells. Mutations in the gene encoding for Bruton’s-tyrosine-kinase (BTK) and the components of the pre-B-cell receptor complex or downstream signaling molecules have been related to this defect in patients with agammaglobulinemia.</p> <p><b>Methods:</b> Iranian patients with congenital agammaglobulinemia were included and the correlation between disease-causing mutations and parameters such as clinical and immunologic phenotypes were evaluated in available patients.</p> <p><b>Results:</b> Out of 87 patients, a molecular investigation was performed on 51 patients leading to identification of 39 cases with BTK (1 novel mutation), 5 cases of µ-heavy chain (3 novel mutations) and 1 case of Igα-deficiencies.</p> <p><b>Conclusion:</b> Although there is no comprehensive correlation between type of responsible <i>BTK</i> mutation and severity of clinical phenotype, our data suggest that BTK-deficient and autosomal recessive agammaglobulinemia patients differ significantly regarding clinical/immunologic characteristics.</p