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T cell functions in infants and children with beta-thalassemia
A S Khalifa1, Z Maged, R Khalil
1Department of Paediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Insights
Beta-thalassemia major patients show lower T cell counts, impacting cell-mediated immunity. This immune dysfunction, particularly in those with infections, suggests increased susceptibility to illness.
Area of Science:
- Immunology
- Hematology
- Pediatrics
Background:
- Beta-thalassemia is a genetic blood disorder affecting hemoglobin production.
- Cell-mediated immunity plays a crucial role in defending against infections.
- Understanding immune status in thalassemia is vital for managing complications.
Purpose of the Study:
- To investigate cell-mediated immunity in children with beta-thalassemia major and trait.
- To identify immune alterations associated with disease severity and complications.
Main Methods:
- Studied 35 children with beta-thalassemia major and 12 with trait.
- Assessed T lymphocyte subsets (OKT3, OKT4, OKT8), spontaneous and total rosettes (E1-RFC, E2-RFC).
- Evaluated migration inhibition factor (MIF) assay and delayed hypersensitivity skin reactions.
Main Results:
- Lower mean T cell counts observed in beta-thalassemia major, not trait.
- Decreased helper/suppressor T cell ratio in patients with hypersplenism.
- Patients with pneumonia or hepatitis had reduced T cells, helper cells, and impaired skin reactions/MIF activity.
Conclusions:
- Cell-mediated immunity is compromised in children with beta-thalassemia major.
- Immune deficits correlate with disease complications like infections.
- Assessing cell-mediated immunity may help identify at-risk thalassemia patients.
Abstract:
Thirty-five infants and children with beta-thalassemia major and 12 with beta-thalassemia trait were studied. Their ages ranged between 6 months and 12 years. Thirty-three were males and 14 females. Spontaneous rosette (E1-RFC), total rosette (E2-RFC), enumeration of T lymphocyte subsets using monoclonal antibodies (OKT3, OKT4, and OKT8), migration inhibition factor (MIF) assay and in vivo delayed hypersensitivity skin reactions were tested. Lower mean T cell population was present in thalassemia major but not the trait. The helper/suppressor ratio was decreased in patients with evidence of hypersplenism. Patients who had suffered from pneumonia or hepatitis manifested lower mean T cell count, depletion of helper cells and decreased helper/suppressor ratio. They also showed depressed delayed cutaneous hypersensitivity and MIF activity. Study of the cell-mediated immunity in patients with thalassemia might be useful to detect those who could be-more susceptible to infections.