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Expansion and Enrichment of Gamma-Delta γδ T Cells from Apheresed Human Product
Published on: September 22, 2021
Good's syndrome with increasing γδ T-lymphocyte subpopulation: A case report
Insights
Good's syndrome, a rare cause of adult-onset immunodeficiency, presents with frequent infections and low immunoglobulins. This case highlights typical immunological findings and an increased gamma delta T-cell receptor subset.
Area of Science:
- Immunology
- Endocrinology
- Oncology
Background:
- Good's syndrome is a rare primary immunodeficiency characterized by thymoma and adult-onset hypogammaglobulinemia.
- It presents with recurrent infections, necessitating investigation of immunoglobulin levels and lymphocyte subsets.
Observation:
- A 56-year-old male presented with a two-year history of recurrent infections.
- Immunological evaluation revealed panhypogammaglobulinemia, B-cell lymphopenia, CD4 lymphopenia, and an inverted CD4/CD8 ratio.
- Chest CT identified a mediastinal mass consistent with thymoma.
Findings:
- The patient exhibited typical immunological markers for Good's syndrome.
- A notable finding was a relative increase in the gamma delta T-cell receptor (TCR) T-lymphocyte subpopulation.
- Intravenous immunoglobulin therapy improved the patient's clinical condition, and the thymoma remained stable over a 3-year follow-up.
Implications:
- This case underscores the importance of considering Good's syndrome in adults with recurrent infections and abnormal immunoglobulin profiles.
- The observed increase in gamma delta T-cells warrants further research to determine its pathogenetic role or reactive nature in Good's syndrome.
- Management with intravenous immunoglobulins can effectively control symptoms and prevent complications.
Introduction:
Good's syndrome is a rare cause of adult-onset immunodeficiency associated with thymoma. Good's syndrome should be considered in patients older than 40 years with the history of frequent infections. An abnormal immunoglobulin profile needs further investigation and flow cytometry which is crucial for establishing the diagno- sis of Good's syndrome.
Case Report:
We present a 56- year-old men with Good's syndrome diagnosed after a two-year history of recurrent infections. Examination of immune status of the patient showed decreased serum levels of all immunoglobulins. Flow cytometry of peripheral blood lym-phocyte revealed markedly reduced peripheral B cells, CD4 T-cell lymphopenia, inverted CD4/CD8 T-cell-ratio 0.37 (CD4--20.82%, CD8--70.7%). Analysis of the subpopulations of T-lymphocytes showed relative increasing γδ T cell receptor (TCR) T lymphocytes. Computed tomography scan of the chest showed a mediastinal mass compatible with thymoma of the diameter of 40 mm. After initiation of intravenous immunoglobulins the patient was in the good clinical condition and without bacterial complications. As the patient refused the operative treatment we continued to control the mediastinal tumor mass which did not increase during a 3-year follow-up.
Conclusion:
The presented patient had a typical immunological finding for Good's syndrome, but also the increase in γδ TCR T-lymphocyte subpopulation for which it is difficult to determine whether this is pathogenetic or secondary reactive event.
