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

被引:13
作者
FISCHER, MB
HAUBER, I
FODINGER, M
WOLF, HM
THON, V
DONATH, P
EIBL, MM
KNOBLER, RM
机构
[1] UNIV VIENNA,INST LAB MED,DIV MOLEC BIOL,VIENNA,AUSTRIA
[2] UNIV VIENNA,DEPT DERMATOL,DIV SPECIAL & ENVIRONM DERMATOL,VIENNA,AUSTRIA
关键词
REVERSE TRANSCRIPTASE POLYMERASE CHAIN REACTION TECHNIQUE; TCR GAMMA CHAIN REARRANGEMENT;
D O I
10.1111/1523-1747.ep12606046
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
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.
引用
收藏
页码:537 / 540
页数:4
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