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T and B lymphocytes in alpha-chain disease.
British Journal of Cancer
|January 1, 1978
Summary
Alpha-chain disease, characterized by abnormal alpha-chain protein, involves a defect in the immune system. Patients exhibit a higher proportion of B lymphocytes and lower T lymphocytes, indicating a B-cell disease with impaired cellular immunity.
Area of Science:
- Immunology
- Hematology
- Gastroenterology
Background:
- Alpha-chain disease is a rare disorder characterized by clinicopathological features, malabsorption, and abnormal alpha-chain protein.
- Understanding the immune system's role is crucial for diagnosing and managing alpha-chain disease.
Purpose of the Study:
- To investigate potential immune system defects in patients diagnosed with alpha-chain disease.
- To characterize the T and B lymphocyte populations and cellular immunity in affected individuals.
Main Methods:
- Enumeration of circulating T and B lymphocytes using the rosette technique and surface immunofluorescence.
- Assessment of cellular immunity through tuberculin skin testing and dinitrochlorobenzene (DNCB) sensitization.
- Quantification of serum immunoglobulins.
Main Results:
- Patients exhibited a significantly higher proportion of circulating B lymphocytes compared to normal controls.
- A lower proportion of circulating T lymphocytes was observed in patients.
- Impaired cellular immunity was indicated by negative tuberculin skin tests and inability to sensitize to DNCB.
Conclusions:
- Alpha-chain disease is identified as a B-cell disorder, specifically of the IgA type.
- The disease is associated with a compromised level of cellular immunity.