title: Large granular lymphocyte expansions in patients with Felty's syndrome: analysis using anti-T cell receptor V beta-specific monoclonal antibodies.
abstract: Felty's syndrome (FS), the association of rheumatoid arthritis (RA) and idiopathic neutropenia, remains an unexplained phenomenon. HLA-DR4 is found in over 90% of cases. Patients with FS may have a T cell lymphocytosis of CD3+CD8+CD57+ large granular lymphocytes (LGL syndrome). In this study of 47 patients with FS, 19% had clear evidence for LGL expansions, while in total 42% had variable evidence for the LGL syndrome using currently available techniques. Of these T cell expansions, 76% were clonal, as demonstrated by Southern blotting and analysis with T cell receptor (TCR) beta chain constant region probes. This technique may fail to detect clonal populations in some patients. Cytofluorographic analysis using antibodies specific for TCR V beta chains identified patients with clonal LGL expansions with results comparable to those obtained with Southern blotting. No evidence for shared V beta usage among expansions from different patients was seen. The role of LGL in RA and FS is currently unclear, but this technique offers a practical and accessible means of identifying patients with LGL expansions, as a starting point for further investigation.
authors.affiliation: Division of Medicine, UMDS, Guy's Hospital, London, UK.
authors.forename: S J
authors.initials: SJ
authors.lastname: Bowman
authors.forename: M
authors.initials: M
authors.lastname: Bhavnani
authors.forename: G C
authors.initials: GC
authors.lastname: Geddes
authors.forename: V
authors.initials: V
authors.lastname: Corrigall
authors.forename: A W
authors.initials: AW
authors.lastname: Boylston
authors.forename: G S
authors.initials: GS
authors.lastname: Panayi
authors.forename: J S
authors.initials: JS
authors.lastname: Lanchbury
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000911:Antibodies, Monoclonal / Q000276:immunology; D005258:Felty Syndrome / Q000276:immunology*; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte / Q000235:genetics; D015804:HLA-DR4 Antigen / Q000235:genetics; D006801:Humans; D007694:Killer Cells, Natural / Q000276:immunology*; D018655:Lymphocyte Count; D016131:Lymphocyte Subsets / Q000276:immunology; D008297:Male; D008875:Middle Aged; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000032:analysis* / Q000235:genetics
chemical_list: D000911:Antibodies, Monoclonal; D015804:HLA-DR4 Antigen; D016693:Receptors, Antigen, T-Cell, alpha-beta