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. 1992 Dec;90(3):390–393. doi: 10.1111/j.1365-2249.1992.tb05856.x

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

W J Monafo 1, S H Polmar 1, S Neudorf 1, A Mather 1, A H Filipovich 1
PMCID: PMC1554576  PMID: 1333922

Abstract

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 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.

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Selected References

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