Novel primary thymic defect with T lymphocytes expressing gamma delta T cell receptor.

Phenotypical and Functional Characterization of Double‐Negative (CD4‐CD8‐) αβ T‐Cell Receptor Positive Cells from an Immunodeficient Patient

“…The DN T cells were also found to be CD57', The CD57 antigen is usually found on NK cells and a small subset of CD8-T cells. However, a high percentage of CD57*^ T cells have been described in patients who have received bone marrow transplantation [35,36] and in patients with other immunodeficiencies [12]. The biological function of CD57 is unknown, but it has been found [35,37] that CD57' T cells are poor IL-2 producers and responders to mitogen stimulation.…”

“…and thymic epiiheiia! cells as described in detail elsewhere [12][13][14], The staining was performed with ihe use of either a three-layer immunoperoxidase method or ihe immunoalkaline phosphatase (APAAP) technique. In all cases, routinely processed formalin-lixed and paraffm-embedded specimens were also available, and Ihese were stained with H&E, May-Grunewald Giemsa, reticulin and periodic acid-Schiff (PAS), Isolation of lymphocytes.…”

New and Old Immunodeficiencies

“…These all have defective T-cell activation with poor prolif- Table 12. Earlv onset erative responses to mitogens and antigens, variable T-cell functional deficiency, and mild to severe susceptibility to infection (60)(61)(62)(63). Two of these patients have a structurally abnormal TCR; one lacked the CD3-{ chain (60), and the second had deficient a/@ TCR-CD3 cells and a great excess of y/6 TCR-CD3 cells (6 1).…”