T cell immunodeficiency in dyskeratosis congenita.
case_report · Level V
Where this comes from
- Record sourced from PubMed, PMID 1580685.
- Also identified by PMC identifier 1793336.
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Abstract
Dyskeratosis congenita has been found to be associated with abnormal immune function. In this study we report a patient with this association. He developed Pneumocystis carinii interstitial pneumonia, and impaired cell mediated immunity was confirmed by the presence of depressed lymphoproliferative responses to in vitro stimulation with mitogen. Enumeration of T cell subsets showed a severely depressed CD4:CD8 ratio (0.38), which is the likely cause for impaired cell mediated immunity. The T cell activation pathway appeared intact, as his T lymphocytes were able to express activation markers (CD25 and HLA-DR) after mitogen stimulation.
Medical subject headings
- Lymphoproliferative Disorders
- Skin Diseases
- T-Lymphocyte Subsets