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@article{837110,
author = {Fischer, MB. and Hauber, I. and Eggenbauer, H. and Thon, Vojtěch and Vogel, E. and Schaffer, E. and Lokaj, J. and Litzman, J. and Wolf, HM. and Mannhalter, JW.},
article_number = {12},
keywords = {HUMAN B CELLS; PROLIFERATIVE RESPONSES; INTERFERON GAMMA; GENE EXPRESSION; INTERLEUKIN 2; HYPOGAMMAGLOBULINEMIA; IL 2; ANTIGEN; DIFFERENTIATION; ABNORMALITIES},
language = {eng},
issn = {0006-4971},
journal = {BLOOD},
title = {A DEFECT IN THE EARLY PHASE OF T-CELL RECEPTOR-MEDIATED T-CELL ACTIVATION IN PATIENTS WITH COMMON VARIABLE IMMUNODEFICIENCY},
volume = {84},
year = {1994}
}
TY - JOUR
ID - 837110
AU - Fischer, MB. - Hauber, I. - Eggenbauer, H. - Thon, Vojtěch - Vogel, E. - Schaffer, E. - Lokaj, J. - Litzman, J. - Wolf, HM. - Mannhalter, JW.
PY - 1994
TI - A DEFECT IN THE EARLY PHASE OF T-CELL RECEPTOR-MEDIATED T-CELL ACTIVATION IN PATIENTS WITH COMMON VARIABLE IMMUNODEFICIENCY
JF - BLOOD
VL - 84
IS - 12
SP - 4234-4241
EP - 4234-4241
SN - 00064971
KW - HUMAN B CELLS
KW - PROLIFERATIVE RESPONSES
KW - INTERFERON GAMMA
KW - GENE EXPRESSION
KW - INTERLEUKIN 2
KW - HYPOGAMMAGLOBULINEMIA
KW - IL 2
KW - ANTIGEN
KW - DIFFERENTIATION
KW - ABNORMALITIES
N2 - Common variable immunodeficiency (CVID) is characterized by an impairment of specific antibody production and a decrease in all or selected Ig isotypes. Abnormalities at the level of the B cells, T cells, and antigen-presenting cells have been described. In the present study, we have focused our attention on T-cell activation in CVID. T cells from 15 of 24 patients failed to respond to recall antigens leg, tetanus toxoid, Escherichia coli), Of these 15 patients, 11 were studied in detail and showed significantly decreased T-cell proliferative responses and/or decreased interleukin-2 and interferon-gamma production on T-cell receptor-mediated stimulation with recall antigens and superantigens (staphylococcal enterotoxins [SEI); however, T-cell response to mitogens (anti-CD3 monoclonal antibody, phytohemagglutinin) was normal. The defect in interleukin-2 and interferon-gamma release on tetanus toroid stimulation could also be documented in purified CD4 T cells of the patients and was present in patients with high and normal CD8 counts alike. Furthermore, patients' T cells failed to mount a significant elevation in free intracellular calcium (Ca++ flux) in response to superantigen, whereas the response to phorbol myristate acetate and ionomycin, bypassing receptor-mediated signaling, was unimpaired. These results indicate a defect in the early phase of T-cell activation after triggering of the T-cell receptor in a significant subgroup of CVID patients.
ER -
FISCHER, MB., I. HAUBER, H. EGGENBAUER, Vojtěch THON, E. VOGEL, E. SCHAFFER, J. LOKAJ, J. LITZMAN, HM. WOLF and JW. MANNHALTER. A DEFECT IN THE EARLY PHASE OF T-CELL RECEPTOR-MEDIATED T-CELL ACTIVATION IN PATIENTS WITH COMMON VARIABLE IMMUNODEFICIENCY. \textit{BLOOD}. 1994, vol.~84, No~12, p.~4234-4241, 7 pp. ISSN~0006-4971.
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