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 toxoid (TT) and T cell clones obtained were compared for their cytokine profile. In the series of PHA-induced or TT-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.
journal_name
Eur J Immunoljournal_title
European journal of immunologyauthors
Amedei A,Romagnani C,Benagiano M,Azzurri A,Fomia F,Torrente F,Plebani A,D'Elios MM,Del Prete Gdoi
10.1002/1521-4141(200106)31:6<1927::aid-immu1927>3keywords:
subject
Has Abstractpub_date
2001-06-01 00:00:00pages
1927-34issue
6eissn
0014-2980issn
1521-4141pii
10.1002/1521-4141(200106)31:6<1927::AID-IMMU1927>3journal_volume
31pub_type
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