Churg Strauss Syndrome (CSS) is a systemic vasculitis in which oligoclonal T cell expansions might be involved in the pathogenesis. Combined analysis of TCR-Vbeta expression profile by flow cytometry and of TCR gene rearrangement by heteroduplex PCR was used to detect and characterize T cell expansions in 8 CSS patients, 10 asthmatics and 42 healthy subjects. In all CSS patients one or two Vbeta families were expanded among CD8+ cells, with an effector memory phenotype apt to populate tissues and inflammatory sites. Heteroduplex PCR showed the presence of one or more clonal TCR rearrangements, which reveals monoclonal or oligoclonal T cells subpopulations. After purification with a Vbeta specific monoclonal antibody, each CD8+/Vbeta+ expanded family showed a single TCR rearrangement, clearly suggestive of monoclonality. All CD8+ expansions were detectable throughout the disease course. TCR-Vbeta expanded or deleted populations were not observed in asthmatic patients. Clonal CD8+/Vbeta+ T cell expansions might be useful as a disease marker.

Clonal CD8+ TCR-Vbeta expanded populations with effector memory phenotype in Churg Strauss syndrome

GUIDA, Giuseppe;VALLARIO, Antonella;BOITA, MONICA;CIRCOSTA, Paola;MARIANI, Sara;HEFFLER, Enrico Marco;BERGIA, Roberta;ROLLA, Giovanni;CIGNETTI, Alessandro
2008

Abstract

Churg Strauss Syndrome (CSS) is a systemic vasculitis in which oligoclonal T cell expansions might be involved in the pathogenesis. Combined analysis of TCR-Vbeta expression profile by flow cytometry and of TCR gene rearrangement by heteroduplex PCR was used to detect and characterize T cell expansions in 8 CSS patients, 10 asthmatics and 42 healthy subjects. In all CSS patients one or two Vbeta families were expanded among CD8+ cells, with an effector memory phenotype apt to populate tissues and inflammatory sites. Heteroduplex PCR showed the presence of one or more clonal TCR rearrangements, which reveals monoclonal or oligoclonal T cells subpopulations. After purification with a Vbeta specific monoclonal antibody, each CD8+/Vbeta+ expanded family showed a single TCR rearrangement, clearly suggestive of monoclonality. All CD8+ expansions were detectable throughout the disease course. TCR-Vbeta expanded or deleted populations were not observed in asthmatic patients. Clonal CD8+/Vbeta+ T cell expansions might be useful as a disease marker.
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Churg Strauss Syndrome; Flow cytometry; Gene rearrangement; T cell Receptor; T-lymphocyte; Aged; Biomarkers; CD8-Positive T-Lymphocytes; Churg-Strauss Syndrome; Clone Cells; Female; Flow Cytometry; Gene Expression; Gene Expression Profiling; Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; Genes, T-Cell Receptor beta; Humans; Immunophenotyping; Male; Middle Aged; Phenotype; Polymerase Chain Reaction; Receptors, Antigen, T-Cell, alpha-beta; T-Lymphocyte Subsets; Immunologic Memory; Immunology; Immunology and Allergy
Giuseppe Guida; Antonella Vallario; Stefania Stella; Monica Boita; Paola Circosta; Sara Mariani; Giuseppina Prato; Enrico Heffler; Roberta Bergia; Antonino Sottile; Giovanni Rolla; Alessandro Cignetti
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2318/57813
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