Objective:

Common variable immunodeficiency (CVID) is a complex inborn error of humoral immunity with complications of 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 aimed to classify Moroccan CVID patients using B-cell immunophenotyping, based on the European classification (EUROclass).

Materials and Methods:

We recruited 20 CVID patients fulfilling established diagnostic standards. After collecting clinical and demographic data, we analyzed B-cell subsets by flow cytometry, grouped patients, and assessed the relationship of each group with clinical manifestations.

Results:

In our cohort, 90% of the patients had a clinical 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 to a lesser extent with granulomatous diseases. Patients had a significant reduction in total, switched memory, marginal zone-like, and plasmablasts, along with a strong 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 had also an expansion of CD21_low B-cells, which was strongly associated with splenomegaly.

Conclusion:

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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