Immunophenotypical alterations in a subset of patients with common variable immunodeficiency (CVID)

Baumert, E.; Wolff-Vorbeck, Guido; Schlesier, Michael; Peter, Hans‐Hartmut

doi:10.1111/j.1365-2249.1992.tb05826.x

Cited by 56 publications

(15 citation statements)

References 47 publications

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“…Other genes we found up-regulated in group I were the genes encoding for the adhesion molecules ICAM-1 and LFA-3. This finding was consistent with previous reports showing an increased expression of these molecules in a subgroup of CVID patients characterized by low CD4:CD8 ratio, increased T cell activation, and splenomegaly (42)(43)(44). Noteworthy was also the differential expression of AF-17 and Ikaros genes that we found in patients of group I and III, which could represent a useful marker for subgroup prediction and assignment.…”

Section: Discussionsupporting

confidence: 81%

Unravelling the Complexity of T Cell Abnormalities in Common Variable Immunodeficiency

Giovannetti

Pierdominici

Mazzetta

et al. 2007

The Journal of Immunology

252

190

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We investigated several phenotypic and functional parameters of T cell-mediated immunity in a large series of common variable immunodeficiency (CVID) patients. We demonstrated that the vast majority of CVID patients presented multiple T cell abnormalities intimately related among them, the severity of which was reflected in a parallel loss of CD4+ naive T cells. A strong correlation between the number of CD4+ naive T cells and clinical features was observed, supporting the subgrouping of patients according to their number of naive CD4+ T lymphocytes. A reduced thymic output and disrupted CD4+ and CD8+ TCR repertoires paralleled the contraction of CD4+ naive T cell pools. The evaluation of activation markers and cytokine production indicated a strong T cell activation that was significantly related to the increased levels of T cell turnover and apoptosis. Finally, discrete genetic profiles could be demonstrated in groups of patients showing extremely diverse T cell subset composition and function. Naive CD4+ T cell levels were significantly associated with the switched memory B cell-based classification, although the concordance between the respective subgroups did not exceed 58.8%. In conclusion, our data highlight the key role played by the T cell compartment in the pathogenesis of CVID, pointing to the need to consider this aspect for classification of this disease.

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Section: Discussionsupporting

confidence: 81%

Unravelling the Complexity of T Cell Abnormalities in Common Variable Immunodeficiency

Giovannetti

Pierdominici

Mazzetta

et al. 2007

The Journal of Immunology

252

190

View full text Add to dashboard Cite

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“…Traditionally, the T-cell abnormalities that characterize CVID included a reduced T-cell count, decreased lymphocyte proliferation in response to mitogens and antigens, defective T-cell signaling, decline of regulatory T cell (Tregs) counts, uncontrolled T-cell polarization, elevated levels of T-cell activation markers, and abnormality in cytokine production secondary to gene polymorphisms [12][13][14][21][22][23][24][25][26].…”

Section: T-cell Abnormalities In Cvidmentioning

confidence: 99%

T-Cell Abnormalities in Common Variable Immunodeficiency

Azizi

Rezaei

Kiaee

et al. 2016

J Investig Allergol Clin Immunol

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Common variable immunodeficiency (CVID) is the most common clinical primary immunodeficiency. It is characterized by a defect in B-cell differentiation to plasma and memory B cells. Moreover, numerous T-cell abnormalities have been reported and include decreased T-cell count and proliferative response, increased T-cell activation and apoptosis, and abnormalities in cytokine production. The aims of this review are to describe phenotypic and functional defects in T cells in CVID patients and to review the literature with respect to the effects of immunoglobulin replacement on the T-cell component in CVID patients. Key words: Common variable immunodeficiency. T cell. Helper T cells. Regulatory T cells. ResumenLa inmunodeficiencia común variable (CVID) es la inmunodeficiencia primaria más frecuente. Se caracteriza por un defecto en la diferenciación de linfocitos B hacia células plasmáticas y linfocitos B memoria. Se han descrito numerosas alteraciones en los linfocitos T de estos pacientes, tales como disminución en el número de linfocitos T y en sus respuestas proliferativas, aumento en la activación de células T y en la apoptosis, así como alteraciones en la producción de citokinas. El objetivo de esta revisión es describir las alteraciones funcionales y fenotípicas de los linfocitos T en los pacientes con CVID y revisar la bibliografía en relación a los efectos que la administración de inmunoglobulinas produce en los linfocitos T de los pacientes con CVID. Palabras clave: Inmunodeficiencia común variable. Células T. Células T helper. Células T reguladoras.

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“…Many studies in CVID are difficult to interpret since abnormalities may result either from altered immune cell populations or from the abnormal function of cells that are present [4]. For example, there is often a dramatic shift in the patients' T cells to a predominant CD45RO ('memory type') phenotype rather than CD45RA ('naive type') cells, as well as the less common occurrence of CD8 predominance [5][6][7][8].…”

Section: Introductionmentioning

confidence: 99%

B cells from a distinct subset of patients with common variable immunodeficiency (CVID) have increased CD95 (Apo-1/fas), diminished CD38 expression, and undergo enhanced apoptosis

Saxon

Keld

Díaz-Sánchez

et al. 1995

Clinical and Experimental Immunology

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SUMMARYWe investigated the role of apoptosis in the differentiation failure of B cells from a selected subpopulation of patients with CVID delineated by B cell surface marker analysis, in vitro IgE response, and molecular markers of B cell VH gene repertoire. These patients had altered display of B cell surface molecules that play a role in apoptosis. The patients' B cells had a 4 5-250-fold increase in CD95 (Apo-l, fas) expression and increased CD95 display on their T cells. CD38, a molecule important in preventing germinal centre B cell apoptosis, was reduced on the patients' B cells. The expression of this molecule was inducible on the CVID lymphocytes with retinoic acid. Increased spontaneous apoptosis in vitro was observed with the patients' B (23%) and T cells (10%) compared with normal cells (13% and 3%, respectively). Stimulation in vitro with IL-4 and CD40 rescued the B cells from apoptosis and allowed for their differentiation. However, IL-4 plus aCD40-driven immunoglobulin production was not quantitatively or qualitatively normal. Failure to overcome apoptosis, a normal step in germinal centre B cell development, may be involved in the lack of differentiation seen in this subset of CVID patients.Keywords common variable immunodeficiency human humoral immunodeficiency antibody deficiency B cell development immunoglobulin production

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Immunophenotypical alterations in a subset of patients with common variable immunodeficiency (CVID)

Cited by 56 publications

References 47 publications

Unravelling the Complexity of T Cell Abnormalities in Common Variable Immunodeficiency

Unravelling the Complexity of T Cell Abnormalities in Common Variable Immunodeficiency

T-Cell Abnormalities in Common Variable Immunodeficiency

B cells from a distinct subset of patients with common variable immunodeficiency (CVID) have increased CD95 (Apo-1/fas), diminished CD38 expression, and undergo enhanced apoptosis

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