"49377" . "Driessen, G. J." . "Noellke, P." . "T-cell receptor V beta skewing frequently occurs in refractory cytopenia of childhood and is associated with an expansion of effector cytotoxic T cells: a prospective study by EWOG-MDS" . "000337229900003" . . . "RIV/00216208:11130/14:10292896" . "Langerak, A. W." . "T-cell receptor V beta skewing frequently occurs in refractory cytopenia of childhood and is associated with an expansion of effector cytotoxic T cells: a prospective study by EWOG-MDS" . . "1"^^ . . "Immunosuppressive therapy (IST), consisting of antithymocyte globulin and cyclosporine A, is effective in refractory cytopenia of childhood (RCC), suggesting that, similar to low-grade myelodysplastic syndromes in adult patients, T lymphocytes are involved in suppressing hematopoiesis in a subset of RCC patients. However, the potential role of a T-cell-mediated pathophysiology in RCC remains poorly explored. In a cohort of 92 RCC patients, we prospectively assessed the frequency of T-cell receptor (TCR) b-chain variable (Vb) domain skewing in bone marrow and peripheral blood by heteroduplex PCR, and analyzed T-cell subsets in peripheral blood by flow cytometry. TCRVb skewing was present in 40% of RCC patients. TCRVb skewing did not correlate with bone marrow cellularity, karyotype, transfusion history, HLA-DR15 or the presence of a PNH clone. In 28 patients treated with IST, TCRVb skewing was not clearly related with treatment response. However, TCRVb skewing did correlate with a disturbed CD4(+)/ CD8(+) T-cell ratio, a reduction in naive CD8(+) T cells, an expansion of effector CD8(+) T cells and an increase in activated CD8(+) T cells (defined as HLA-DR+, CD57(+) or CD56(+)). These data suggest that T lymphocytes contribute to RCC pathogenesis in a proportion of patients, and provide a rationale for treatment with IST in selected patients with RCC." . "11130" . "Fischer, A." . "7"^^ . "van der Velden, V. H. J." . . "Hasle, H." . "Yoshimi, A." . "I" . . . . "Beverloo, H. B." . "van den Heuvel-Eibrink, M. M." . . "Blood Cancer Journal" . . "Locatelli, F." . . . . "Immunosuppressive therapy (IST), consisting of antithymocyte globulin and cyclosporine A, is effective in refractory cytopenia of childhood (RCC), suggesting that, similar to low-grade myelodysplastic syndromes in adult patients, T lymphocytes are involved in suppressing hematopoiesis in a subset of RCC patients. However, the potential role of a T-cell-mediated pathophysiology in RCC remains poorly explored. In a cohort of 92 RCC patients, we prospectively assessed the frequency of T-cell receptor (TCR) b-chain variable (Vb) domain skewing in bone marrow and peripheral blood by heteroduplex PCR, and analyzed T-cell subsets in peripheral blood by flow cytometry. TCRVb skewing was present in 40% of RCC patients. TCRVb skewing did not correlate with bone marrow cellularity, karyotype, transfusion history, HLA-DR15 or the presence of a PNH clone. In 28 patients treated with IST, TCRVb skewing was not clearly related with treatment response. However, TCRVb skewing did correlate with a disturbed CD4(+)/ CD8(+) T-cell ratio, a reduction in naive CD8(+) T cells, an expansion of effector CD8(+) T cells and an increase in activated CD8(+) T cells (defined as HLA-DR+, CD57(+) or CD56(+)). These data suggest that T lymphocytes contribute to RCC pathogenesis in a proportion of patients, and provide a rationale for treatment with IST in selected patients with RCC."@en . "Zwaan, C. M." . "4" . "Aalbers, A. M." . "Dworzak, M." . "De Moerloose, B." . "Star\u00FD, Jan" . "10.1038/bcj.2014.28" . . "22"^^ . "Pieters, R." . . "GB - Spojen\u00E9 kr\u00E1lovstv\u00ED Velk\u00E9 Brit\u00E1nie a Severn\u00EDho Irska" . . "van Dongen, J. J. M." . "Niemeyer, C. M." . "2044-5385" . . "Baumann, I." . "neuveden" . "Zecca, M." . "Schmugge, M." . "http://dx.doi.org/10.1038/bcj.2014.28" . . "[E51519C5F2A4]" . "T-cell receptor V beta skewing frequently occurs in refractory cytopenia of childhood and is associated with an expansion of effector cytotoxic T cells: a prospective study by EWOG-MDS"@en . "population; cyclosporine-a; clinical-significance; autologous lymphocytes; immunosuppressive therapy; aplastic-anemia; bone-marrow; antithymocyte globulin; risk myelodysplastic syndrome; paroxysmal-nocturnal hemoglobinuria"@en . . "T-cell receptor V beta skewing frequently occurs in refractory cytopenia of childhood and is associated with an expansion of effector cytotoxic T cells: a prospective study by EWOG-MDS"@en . "Goehring, G." . . "RIV/00216208:11130/14:10292896!RIV15-MSM-11130___" . .