1887
2 - Second Qatar Allergy Conference
  • ISSN: 0253-8253
  • EISSN: 2227-0426

Abstract

Common variable immunodeficiency (CVID) is a complex inborn error of humoral immunity with complications of both infectious and non-infectious origins. Classifications of CVID patients provide a clearer understanding of the pathogenesis, prediction, and management of non-infectious complications. This study aims to classify Moroccan CVID patients based on the European classification (EUROclass).

We recruited 20 CVID patients meeting standard diagnostic criteria (5-6). After collecting clinical and demographic data, we used flow cytometry to analyze B-cell subsets and group patients and assess the relation of each group with clinical manifestations.

90% of the patients in our cohort study had a history of respiratory infections. The non-infectious manifestations included splenomegaly, autoimmunity, lymphadenopathy, and granulomatous diseases diagnosed in 50%, 45%, 40%, and 25% of patients, respectively. We observed significant co-occurrence of splenomegaly with autoimmunity and granulomatous diseases to a lesser extent. Patients had a significant reduction in total, switched memory, marginal zone-like, plasma blasts, and a substantial increase in the percentage of activated B cells, suggesting a defect in the late phases of B-cell differentiation. This condition was linked with an increased occurrence of splenomegaly and granulomatous affections. Besides, patients also had an expansion of CD21low B-cells, which was strongly associated with splenomegaly.

The classification of the first Moroccan cohort of CVID patients showed agreement with previous results. It suggests the possibility of adopting this approach on a global scale for better diagnosis and follow-up of CVID patients.

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2023-05-21
2024-03-28
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References

  1. Hammarstrom, L., I. Vorechovsky, and D. Webster, Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID). Clin Exp Immunol, 2000. 120:(2): p. 225-31.
    [Google Scholar]
  2. Modell, V., et al., Global overview of primary immunodeficiencies: a worldwide report from Jeffrey Modell Centers focused on diagnosis, treatment, and discovery. Immunol Res, 2014. 60:(1):p. 132-44.
    [Google Scholar]
  3. Wehr, C., et al., The EUROclass trial: defining subgroups in common variable immunodeficiency. Blood, 2008. 111:(1): p. 77-85.
    [Google Scholar]
  4. Bousfiha, A., et al., The 2022 Update of IUIS Phenotypical Classification for Human Inborn Errors of Immunity. J Clin Immunol, 2022.
    [Google Scholar]
  5. Aghamohammadi, A., et al., Clinical and immunological features of 65 Iranian patients with common variable immunodeficiency. Clin Diagn Lab Immunol, 2005. 12:(7): p. 825-32.
    [Google Scholar]
  6. Warnatz, K., et al., Severe deficiency of switched memory B cells (CD27(+)IgM(-)IgD(-)) in subgroups of patients with common variable immunodeficiency: a new approach to classify a heterogeneous disease. Blood, 2002. 99:(5): p. 1544-51.
    [Google Scholar]
  7. Yazdani, R., et al., Comparison of various classifications for patients with common variable immunodeficiency (CVID) using measurement of B-cell subsets. Allergol Immunopathol (Madr), 2017. 45:(2): p. 183-192.
    [Google Scholar]
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