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Published on: September 20, 2024
Clinical and laboratory aspects of common variable immunodeficiency
Cristina M Kokron1, Paolo R Errante, Myrthes T Barros
1Serviço de Imunologia, Av. Dr. Anéas de Carvalho Aguilar, 155, 8 andar, bloco 3, 05403-010 São Paulo, SP, Brazil.
Insights
Common variable immunodeficiency (CVID) impairs antibody production, leading to infections. Brazilian CVID patients exhibit reduced T and B cells, increased lymphocyte apoptosis, and lower activation molecule expression, impacting cellular immunity.
Area of Science:
- Immunology
- Clinical Medicine
Background:
- Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by impaired antibody production, recurrent infections, autoimmune disorders, and increased cancer risk.
- CVID can involve cellular immune disturbances, including altered lymphocyte counts, reduced proliferation, cytokine dysregulation, and deficient cell-surface molecule expression.
Purpose of the Study:
- To investigate immunological disturbances in Brazilian CVID patients.
- To correlate these immunological findings with clinical features and compare them with existing literature.
Main Methods:
- Analysis of T and B lymphocyte counts in peripheral blood.
- Assessment of lymphocyte apoptosis susceptibility following activation.
- Evaluation of activation molecule expression (CD25, CD69, CD40L, CD70).
- Measurement of cytokine synthesis (IL-4, IL-5).
Main Results:
- Brazilian CVID patients showed decreased T and B lymphocyte counts.
- Lymphocytes from CVID patients were more susceptible to apoptosis after activation.
- Reduced expression of activation molecules (CD25, CD69, CD40L, CD70) was observed.
- Decreased synthesis of IL-4 and IL-5 was noted in CVID patients compared to controls.
Conclusions:
- Increased lymphocyte apoptosis following activation may explain reduced activation molecule expression, decreased Th2 cytokine synthesis, and lower circulating T and B cell counts in these CVID patients.
- These immunological findings contribute to understanding the pathophysiology of CVID and its clinical manifestations.
Abstract:
Common variable immunodeficiency (CVID) is an immunological disorder characterized by defective antibody production, recurrent infections, most notably of the respiratory tract, autoimmune phenomena and cancer. Some CVID patients may also present disturbances of the cellular immune response such as a decrease in the number and proportion of different lymphocyte populations, diminished lymphoproliferative response to mitogens and antigens, altered production of cytokines, and deficient expression of cell-surface molecules. Most Brazilian CVID patients included in this study show a decrease in T and B lymphocyte counts in the peripheral blood. Furthermore, their lymphocytes are more susceptible to apoptosis following activation than normal individuals, and they have a decrease in the expression of activation molecules like CD25, CD69, CD40L and CD70. Moreover, they show a decreased synthesis of IL-4 and IL-5 in comparison with normal individuals. The increase in susceptibility to apoptosis following activation, may also be responsible for the decrease in the expression of activation molecules and CD40L, decrease in Th2 cytokines synthesis, and in the number of T and B circulating cells. In this study we discuss some of these immunological disturbances correlating them to the patients' clinical features and comparing our patients' findings to the literature.
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