X-linked agammaglobulinemia (XLA) is a primary immunodeficiency of the B-cell compartment caused by a defective gene encoding for the tyrosine kinase (btk) essential for B cell differentiation. Affected males undergo recurrent pyogenic infections and deficient immunoglobulin production. Peripheral blood T cells from 6 XLA patients and 6 matched healthy controls were stimulated with either PHA or tetanus toroid (TT) and T cell clones obtained were compared for their cytokine profile. In the series of PHA-induced or rr-specific CD4(+) T cell clones derived from XLA patients, the Th1 profile was predominant (63 and 65%, respectively). Upon stimulation with TT, the proportion of activated T cells from XLA that expressed the IFN-gamma -associated LAG-3 activation molecule was higher than in control T cells (51 vs. 25%), whereas the expression of the IL-4-associated CD30 molecule was lower (5 vs. 21%). in a cohort of 31 XLA patients, plasma levels of soluble (s)LAG-3 and sCD30, chosen as indirect indicators of the Th1/Th2 activity in vivo, were significantly higher and lower, respectively, than those measured in 31 healthy controls. Likewise, plasma levels of interferon-inducible protein 10 and of macrophage-derived chemokine in XLA patients were significantly higher and lower, respectively,than in healthy controls.
Amedei, A., Romagnani, C., Benagiano, M., Azzurri, A., Fomia, F., Torrente, F., et al. (2001). Preferential Th1 profile of T helper cell responses in X-linked (Bruton's) agammaglobulinemia. EUROPEAN JOURNAL OF IMMUNOLOGY, 31(6), 1927-1934 [10.1002/1521-4141(200106)31:6<1927::aid-immu1927>3.0.co;2-d].
Preferential Th1 profile of T helper cell responses in X-linked (Bruton's) agammaglobulinemia
D'Elios, M. M.;
2001-01-01
Abstract
X-linked agammaglobulinemia (XLA) is a primary immunodeficiency of the B-cell compartment caused by a defective gene encoding for the tyrosine kinase (btk) essential for B cell differentiation. Affected males undergo recurrent pyogenic infections and deficient immunoglobulin production. Peripheral blood T cells from 6 XLA patients and 6 matched healthy controls were stimulated with either PHA or tetanus toroid (TT) and T cell clones obtained were compared for their cytokine profile. In the series of PHA-induced or rr-specific CD4(+) T cell clones derived from XLA patients, the Th1 profile was predominant (63 and 65%, respectively). Upon stimulation with TT, the proportion of activated T cells from XLA that expressed the IFN-gamma -associated LAG-3 activation molecule was higher than in control T cells (51 vs. 25%), whereas the expression of the IL-4-associated CD30 molecule was lower (5 vs. 21%). in a cohort of 31 XLA patients, plasma levels of soluble (s)LAG-3 and sCD30, chosen as indirect indicators of the Th1/Th2 activity in vivo, were significantly higher and lower, respectively, than those measured in 31 healthy controls. Likewise, plasma levels of interferon-inducible protein 10 and of macrophage-derived chemokine in XLA patients were significantly higher and lower, respectively,than in healthy controls.File | Dimensione | Formato | |
---|---|---|---|
agamma.pdf
non disponibili
Tipologia:
PDF editoriale
Licenza:
NON PUBBLICO - Accesso privato/ristretto
Dimensione
1.01 MB
Formato
Adobe PDF
|
1.01 MB | Adobe PDF | Visualizza/Apri Richiedi una copia |
I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
https://hdl.handle.net/11365/1220560