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Phenotypical and Functional Characterization of Double‐Negative (CD4 ‐ CD8 ‐ ) αβ T‐Cell Receptor Positive Cells from an Immunodeficient Patient
Author(s) -
ILLUM N.,
RALFKIAER E.,
PALLESEN G.,
GEISLERt C.
Publication year - 1991
Publication title -
scandinavian journal of immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.934
H-Index - 88
eISSN - 1365-3083
pISSN - 0300-9475
DOI - 10.1111/j.1365-3083.1991.tb01587.x
Subject(s) - phenotype , microbiology and biotechnology , cd8 , cell , cytotoxic t cell , biology , chemistry , immunology , genetics , antigen , in vitro , gene
We have characterized CD4 ‐ CD8 ‐ double‐negative (DN) αβTCR + T cells from a patient with immunodeficiency, lymphocytosis, lymphadenopathy, and hepatosplenomegaly. The majority of peripheral blood lymphocytes were DN αβTCR + T cells as evalualed by FACS and biochemical analysis. The DNT cells showed the following phenotype: αβTCR + , γδTCR ‐ , CD2 + , CD3 + , CD57 + , and HLA‐DR + . Both southern blot analysis of TCR genes and F ACS analysis applying a panel of Vβ and Vα monoclonal antibodies (MoAbs) indicated a polyclonal T‐cell expansion. Thymic biopsy showed normal histology, whereas lymph node biopsy samples showed altered histological and immunohistoiogical patterns with markedly expanded paracortical areas containing the DN T ceils of the same phenotype as found in peripheral blood T cells. In functional studies, the DN T cells showed a profoundly reduced proliferative response upon stimulation with mitogens as well as MoAbs against the TCR /CD3 complex. CD2. and CD28, respectively, Addition of exogenous interleukiri‐2 (IL‐2) only minimally augmented the proliferative response. In contrast, the addition of a combination of Ca 2+ ionophore and phorbol 12‐myristate 13‐acetaie (PMA) restored the proliferative response of the DM T cells to almost normal levels. This observation strongly suggests that the protein kinase C activity of the DN T cells was intact, but that the normal mechanism for transmembrane signal transduction was impaired in these unusual DN T cells.