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.
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Kuipers, J.G., Jacobs, R., Kemper, A. et al. TCR1+ large granular lymphocyte proliferation in rheumatoid arthritis. Rheumatol Int 14, 163–168 (1994). https://doi.org/10.1007/BF00579702
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DOI: https://doi.org/10.1007/BF00579702