ISSN:
1437-160X
Keywords:
Tγ-lymphoproliferative syndrome
;
Large granular lymphocytes
;
Rheumatoid arthritis
;
α/β T cell receptor (TCR2)
;
γ/δ TCR1+
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract The Tγ-lymphoproliferative syndrome is characterized by a proliferation of large granular lymphocytes (LGL). It is often associated with neutropenia, and in 30% of cases with rheumatoid arthritis (RA). Phenotypic analysis has demonstrated that in most cases of RA with Tγ-proliferative disease, the LGL represent T cells with a clonal rearrangement of the α/β T cell receptor (TCR2). Here, three patients with γ/δ TCR1+ LGL proliferation suffering from long-standing arthritis and neutropenia are described. The first patient with RA showed an expansion of a heterogeneous CD2+ CD16+ CD56- LGL population, of which 30% coexpressed TCR1 with Vδ1 rearrangement. The second patient with ankylosing spondylitis and RA was suffering from proliferation of TCR1+ (Vγ9-, Vδ1-), CD2+ CD16- CD56- LGL with low coexpression of CD8. The third patient with RA was suffering from a proliferation of TCR1+ (Vδ1+, Vγ9-) CD4- CE8- CD16- CD56- lymphocytes. On the basis of these unusual findings, the pathogenetic role of TCR1+ T cells in RA is discussed.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00579702
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