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Published on: September 7, 2018
Thymic output changes in children with clinical findings signaling a probable primary immunodeficiency
Neslihan Karaca1, Elif Azarsız2, Sanem Eren Akarcan1
1Departments of Pediatric Immunology, Ege University Faculty of Medicine, İzmir, Turkey.
Insights
Evaluating thymic output using CD31+ recent thymic emigrant (RTE) cells can rapidly assess T-cell immune dysfunction in children with primary immunodeficiency. This method may serve as an alternative to TRECs for diagnosing combined immunodeficiencies.
Area of Science:
- Immunology
- Pediatrics
- Flow Cytometry
Background:
- Primary immunodeficiencies (PIDs) encompass a range of disorders affecting the immune system.
- T-cell immunodeficiencies are particularly severe, necessitating thorough thymic maturation evaluation.
- Assessing thymic output is crucial for diagnosing and managing PIDs in children.
Purpose of the Study:
- To evaluate the utility of the T-cell surface molecule CD31 for assessing thymic output in children with suspected primary immunodeficiency.
- To investigate T-cell subpopulations, including naive, memory, and recent thymic emigrant (RTE) cells, in this patient cohort.
- To explore the correlation between CD31 expression on RTE cells and specific immunodeficiency diagnoses.
Main Methods:
- A cohort of 66 children with clinical findings suggestive of PID were studied.
- Flow cytometry was used to analyze T-cell subpopulations, specifically naive (CD4+CD45RA+) and recent thymic emigrant (CD4+CD45RA+CD31+) cells.
- Clinical and laboratory data were collected to determine diagnoses and patient history, including cardiac surgery.
Main Results:
- Combined immunodeficiency and humoral immunodeficiency were the most frequent diagnoses.
- Significantly lower percentages and absolute counts of naive T-cells and absolute counts of CD31+ RTE cells were observed in the combined immunodeficiency group.
- Patients with a history of cardiac surgery showed lower percentages of naive T-cells and RTE cells.
Conclusions:
- Flow cytometric evaluation of CD31+ thymic naive RTE cells offers rapid clinical information regarding T-cell immune dysfunction.
- CD4+CD45RA+CD31+ RTE cells show potential as an alternative to TRECs in diagnosing combined immunodeficiencies.
- This approach aids in the comprehensive assessment of primary immunodeficiencies in pediatric patients.
Abstract:
Karaca N, Azarsız E, Akarcan SE, Aksu G, Kütükçüler N. Thymic output changes in children with clinical findings signaling a probable primary immunodeficiency. Turk J Pediatr 2019; 61: 885-894. Thymic maturation evaluation is inevitable for patients with clinical and laboratory findings for a primary immunodeficiency, as the T cellimmunodeficiencies are the most severe type. In this study, we aimed to show the usage of T cell surface molecule `CD31` for the evaluation of thymic output in patients (n: 66) with a large spectrum of findings signing a probable primary immunodeficiency. Besides the classical clinical and laboratory approach for these patients, T cell subpopulations as naive, memory, recent thymic emigrant cells were also investigated. The humoral immunodeficiency (34.8%), combined immunodeficiency (34.8%) and cardiopathy (7.6%) were the most frequent diagnosis groups. CD4+CD45RA+ naive T-cells percentages (p: 0.011) and absolute counts (p: 0.004) and absolute CD4+CD45RA+CD31+ RTE (recent thymic emigrant) cell counts (p: 0.007) were significantly lower in combined immunodeficiency group. Naive T-cells (p: 0.037) and RTE cells (p: 0.032) were also lower in patients who had cardiac surgery in the past. In conclusion, flow cytometric CD31+thymic naive RTE cell evaluation may provide rapid clinical information especially on T-cell immune dysfunction and CD4+CD45RA+CD31+ RTE cells may be used as an alternative to TRECs in the diagnosis of combined immunodeficiencies.
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