title: Lymphoproliferative disease of granular lymphocytes with T-cell receptor gamma delta-positive phenotype: restricted usage of T-cell receptor gamma and delta subunit genes.
abstract: 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.
authors.affiliation: Second Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Makishima
authors.forename: Fumihiro
authors.initials: F
authors.lastname: Ishida
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Saito
authors.forename: Naoaki
authors.initials: N
authors.lastname: Ichikawa
authors.forename: Yayoi
authors.initials: Y
authors.lastname: Ozaki
authors.forename: Susumu
authors.initials: S
authors.lastname: Ito
authors.forename: Masao
authors.initials: M
authors.lastname: Ota
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Katsuyama
authors.forename: Kendo
authors.initials: K
authors.lastname: Kiyosawa
mesh_terms: 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*
chemical_list: 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