A hereditary immunodeficiency characterized by CD8 T lymphocyte deficiency and impaired lymphocyte activation

SUMMARY An unusual form of severe combined immunodeficiency in children from two different families was associated with absence of CD8+ T lymphocytes and normal numbers of CD4+ T lymphocytes that did not respond to stimulation by non‐specific mitogens, specific antibodies against the T cell receptor...

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Bibliographic Details
Published in:Clinical and experimental immunology 1992-12, Vol.90 (3), p.390-393
Main Authors: MONAFO, W. J., POLMAR, S. H., NEUDORF, S., MATHER, A., FILIPOVICH, A. H.
Format: Article
Language:English
Subjects:
Blood Cells - immunology
Calcium Channels - physiology
CD3 Complex - pharmacology
CD8 Antigens - analysis
CD8 Antigens - blood
CD8+ T lymphocyte
Family Health
Female
Fluorescent Antibody Technique
Humans
Infant
lymphocyte activation
Lymphocyte Activation - drug effects
Lymphocyte Depletion
lymphocyte development
Lymphoid Tissue - immunology
Male
Phorbol Esters - pharmacology
severe combined immunodeficiency
Severe Combined Immunodeficiency - genetics
T-Lymphocytes - immunology
T-Lymphocytes - ultrastructure
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Summary:SUMMARY An unusual form of severe combined immunodeficiency in children from two different families was associated with absence of CD8+ T lymphocytes and normal numbers of CD4+ T lymphocytes that did not respond to stimulation by non‐specific mitogens, specific antibodies against the T cell receptor or specific antigens. The defect in the CD4+ cells was bypassed by activating agents which are independent of the T cell receptor. The combination of an activation defect and selective depletion of CD8+ T lymphocytes suggests that the defective pathway is important in the differentiation of immature thymocytes as well as the proliferation of mature lymphocytes.
ISSN:0009-9104
1365-2249
DOI:10.1111/j.1365-2249.1992.tb05856.x