DEFECTIVE TCR SURFACE EXPRESSION ASSOCIATED WITH IMPAIRED TCR BETA-CHAIN ASSEMBLY IN A PATIENT WITH CUTANEOUS T-CELL LYMPHOMA

Authors

FISCHER MB. HAUBER I. FODINGER M. WOLF HM. THON Vojtěch DONATH P. EIBL MM. KNOBLER RM,

Year of publication 1995
Type Article in Periodical
Magazine / Source JOURNAL OF INVESTIGATIVE DERMATOLOGY
MU Faculty or unit

Faculty of Medicine

Citation
Field Immunology
Keywords RECEPTOR COMPLEX; LYMPHOCYTES T; ANTIGEN; SEQUENCE; SUBUNITS; T cell lymphoma
Description We report on a patient with cutaneous T-cell lymphoma (CTCL) of long-standing duration. Phenotypic analysis of his peripheral blood mononuclear cells revealed an increased CD4(+) T-helper subset and a decreased CD8(+) cytotoxic T-cell population. Eighty-three to ninety-three percent of the patient's CD4(+) T cells in the peripheral blood and 70% of the CD4(+) T cells in the lesional skin lacked surface expression of the TCR/CD3 complex and showed a clonal rearrangement pattern of the TCR gamma-chain gene (V11-J1/J2). The lack in TCR surface expression correlated with defective assembly of the TCR beta-chain. Although mRNA for the TCR constant region beta 1 was found in the patient's purified CD4(+)TCR(-)CD3(-) T cells, no intracytoplasmic TCR beta protein was detectable. In contrast, the patient's purified CD4(+)TCR(-)CD3(-) T cells not only expressed mRNA specific for the TCR alpha-chain and for all CD3 chains, but intracytoplasmic TCR alpha and CD3 epsilon proteins could also be found. The lack of TCR beta protein clearly explains the defective surface expression of the TCR/CD3 complex in the patient's malignant T cells.

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