Application of a multiprobe RNase protection assay and junctional sequences to define V beta gene diversity in Sezary syndrome.
Nineteen patients with mycosis fungoides/Sezary syndrome (MF/SZ), a malignancy of the mature helper T-cell phenotype (CD4+TCR alpha beta+), were screened for clonotypic V beta expansions in peripheral blood with a multiprobe RNase protection assay. A different predominant V beta gene was identified in 9 of 14 patients with high peripheral blood CD4/CD8 ratios, whereas 4 of these patients showed T-cell expansions expressing V beta genes other than those included in the assay. In contrast, five patients with few, if any, malignant cells in the circulation had V beta expression levels similar to that in normal peripheral blood. A unique V-D-J sequence was found for each highly expressed V beta gene, thereby documenting monoclonality of the expanded T-cell populations. Polymerase chain reaction (PCR) primers specific for the D-J beta junction accurately identified the corresponding malignant clonotype in peripheral blood. The diverse TCR V beta gene usage found in these MF/SZ patients suggests that T-cell receptor (TCR) specificity has no bearing on this disease.
Kono
D H
DH
Department of Immunology, Scripps Research Institute, La Jolla, California.
Baccala
R
R
Balderas
R S
RS
Kovac
S J
SJ
Heald
P W
PW
Edelson
R L
RL
Theofilopoulos
A N
AN
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001483:Base Sequence; D003001:Cloning, Molecular; D005260:Female; D015972:Gene Expression Regulation, Neoplastic; D005821:Genetic Techniques; D014644:Genetic Variation; D006801:Humans; D008297:Male; D008875:Middle Aged; D008969:Molecular Sequence Data; D009182:Mycosis Fungoides / Q000235:genetics; D015345:Oligonucleotide Probes / Q000235:genetics; D016133:Polymerase Chain Reaction; D011948:Receptors, Antigen, T-Cell / Q000235:genetics; D012260:Ribonucleases; D012751:Sezary Syndrome / Q000097:blood / Q000235:genetics*; D012878:Skin Neoplasms / Q000097:blood / Q000235:genetics*
D015345:Oligonucleotide Probes; D011948:Receptors, Antigen, T-Cell; D012260:Ribonucleases