Lymphoproliferative disease of granular lymphocytes with T-cell receptor gamma delta-positive phenotype: restricted usage of T-cell receptor gamma and delta subunit genes.
Lymphoproliferative disease of granular lymphocytes (LDGL) is characterized by more than 0.5 x 109/L of proliferating granular lymphocytes in the peripheral blood. Because of its rarity, the characteristics of LDGL with T-cell receptor (TCR) gammadelta phenotype (gammadeltaT-LDGL) have not yet been identified. This report describes the clinical, hematological, and immunological findings of four patients with this disease. In two cases, the clinical course was indolent and the other two patients required various therapies. The cells had a common immunophenotype: CD3+, CD4-, CD16+, CD56-, CD57-, CD122-, TCR-gammadelta+, and three were CD8-positive. The immunopurified TCR-gammadelta cells from the patients expressed only Vgamma9 and Vdelta1. Spectratyping and sequencing showed mono- or oligoclonality for TCRgamma and TCRdelta subunit genes. Soluble Fas ligand in sera was significantly elevated in all patients. These findings suggest that gammadeltaT-LDGL qualifies as a distinct disease entity.
Makishima
Hideki
H
Second Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
Ishida
Fumihiro
F
Saito
Hiroshi
H
Ichikawa
Naoaki
N
Ozaki
Yayoi
Y
Ito
Susumu
S
Ota
Masao
M
Katsuyama
Yoshihiko
Y
Kiyosawa
Kendo
K
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000920:Antibody-Dependent Cell Cytotoxicity; D015703:Antigens, CD / Q000032:analysis; D002869:Chromosome Aberrations; D002896:Chromosomes, Human, Pair 6 / Q000648:ultrastructure; D002999:Clone Cells / Q000473:pathology; D003602:Cytotoxicity, Immunologic; D053222:Fas Ligand Protein; D005260:Female; D016694:Gene Rearrangement, delta-Chain T-Cell Antigen Receptor; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006801:Humans; D016130:Immunophenotyping; D007621:Karyotyping; D007694:Killer Cells, Natural / Q000276:immunology; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008232:Lymphoproliferative Disorders / Q000097:blood / Q000235:genetics* / Q000473:pathology; D008297:Male; D008562:Membrane Glycoproteins / Q000097:blood; D016133:Polymerase Chain Reaction; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics*; D016176:T-Lymphocyte Subsets / Q000473:pathology*
D015703:Antigens, CD; C505661:FASLG protein, human; D053222:Fas Ligand Protein; D008562:Membrane Glycoproteins; D016692:Receptors, Antigen, T-Cell, gamma-delta; D007770:L-Lactate Dehydrogenase