Existence of activated and memory CD4+ T cells in peripheral blood and their skin infiltration in CD8 deficiency - PubMed (original) (raw)

Case Reports

Existence of activated and memory CD4+ T cells in peripheral blood and their skin infiltration in CD8 deficiency

K Katamura et al. Clin Exp Immunol. 1999 Jan.

Abstract

CD8 deficiency is a rare primary immunodeficiency caused by the defect of a tyrosine kinase, ZAP-70, which transduces signals from the T cell receptor. We report here a case of CD8 deficiency, having CD4+ T cells with a unique phenotype. The patient's T cells did not respond to anti-CD3 stimulation in vitro, suggesting that they were naive. However, many CD4+ T cells with activated and memory phenotypes, which expressed CD45RO+, HLA-DR+ and CD25+, were present in the peripheral blood, and these cells accumulated in the perivascular area of his infiltrative erythematous skin lesions. The patient's T cells could be activated by a high concentration of phytohaemagglutinin (PHA), indicating the presence of an alternate signalling pathway which bypasses ZAP-70 and activates CD4+ T cells in vivo. The origin and role of activated CD4+ T cells in the pathogenesis involved in the skin lesions are discussed.

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Figures

Fig. 1

Flow cytometric analysis of patient's peripheral T cells. Patient's peripheral blood mononuclear cells (PBMC) were stained with FITC-labelled anti-CD3 and PE-labelled anti-CD4 or anti-CD8. Stained cells were analysed as described in Patient and Methods.

Fig. 2

Western blotting of ZAP-70 protein. Patient's T cells, normal control's T cells and Jurkat cells were lysed and ZAP-70 protein was analysed with Western blotting as described in Patient and Methods.

Fig. 3

The patient's skin lesions on his face and extremities.

Fig. 4

(See next page.) Immunohistochemical analysis of patient's skin lesion. (a) Haematoxylin–eosin stain of a skin lesion. The perivascular area is magnified in right upper portion. (b) Immunohistochemical analysis of infiltrated cells. Cells were stained with indicated antibodies. Upper left: anti-CD3; upper middle, anti-CD4; upper right, anti-CD8; lower left, anti-HLA-DR; lower middle, anti-CD45RO.

Fig. 4

Fig. 5

Three-colour analysis of peripheral CD4+ T cells from the ZAP-70-deficient patient. CD4+ T cells were gated by staining with PE-Cy5-labelled anti-CD4, and analysed with FITC-conjugated and PE-conjugated antibodies. Vertical and horizontal axes represent logarithm of fluorescence intensity, and the percentages of cells in each region are indicated.

Fig. 6

Proliferative responses of control's (▪) and patient's (□) peripheral blood mononuclear cells (PBMC) to various concentrations of phytohaemagglutinin (PHA). Data represent the mean of triplicate determinations. S.d. values are within 10% of each data point.

References

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Existence of activated and memory CD4+ T cells in peripheral blood and their skin infiltration in CD8 deficiency - PubMed (original) (raw)