Recent thymic emigrants, T regulatory cells, and BAFF level in children with X-linked agammaglobulinaemia in association with chronic respiratory disease

Background: X-linked agammaglobulinaemia (XLA) is a genetic disorder affecting B cell maturation, which is characterised by a low number of B cells, agammaglobulinaemia and increased susceptibility to a variety of bacterial infections. This study was performed to assess T cell subpopulations in a group of children with XLA in association with chronic respiratory disease (CRD). Methods: Numbers of T cell subpopulations (CD3+, CD4+, CD8+, CD3+DR+, naïve, memory, recent thymic emigrants (RTE), regulatory T cells, follicular T helpers) were measured by eight-colour flow cytometry in 22 XLA patients and 50 controls. BAFF level was measured by ELISA. Results: XLA patients with CRD had a significantly lower percentage of RTE numbers and Tregs, while significantly higher absolute counts of lymphocytes, CD3+, CD8+, CD3+DR+ and CD4+CD45RO+ T cells were detected as compared with healthy controls. In patients with XLA without CRD, the number of follicular T helper cells was altered significantly (percentage and absolute), as compared with healthy controls. Additionally, they had significantly higher counts (percentage and absolute) of CD4+CD45RA+ cells and lower percentage of CD4+CD45RO+ cells in comparison with healthy controls. Conclusions: Our study affords new information concerning CRD and T cell subsets that differentiate or are maintained in the absence of B cells in children with XLA. T cell's homeostasis depends on the presence of chronic respiratory disease that may be caused by the delay in diagnosis (AU)

No disponible

Changes in BAFF/APRIL Levels in a 2-Year-Old Girl with Kawasaki Disease Refractory to Intravenous Immunoglobulin Therapy

No disponible

Factor activador de célula B perteneciente a la familia del TNF (BAFF): Blanco terapéutico por su papel en autoinmunidad / B-CELL activating factor of the tumor necrosis factor family (BAFF): A potential therapeutic target in autoimmunity

Factor activador de célula B perteneciente a la familia del TNF (BAFF)es una proteína que ejerce importantes funciones reguladoras en la supervivencia, maduración y diferenciación de células B, así como en el desarrollo de órganos linfoides, a través de su interacción con distintos receptores. En el laboratorio, utilizando varios modelos animales se han caracterizado algunas de sus propiedades, sus mecanismos de señalización y la participación en la respuesta inmunológica. Por otro lado se ha demostrado en animales y humanos su expresión alterada en diversos contextos como enfermedades autoinmunes, neoplasias linfoides y susceptibilidad a infecciones. La sobreproducción y la distribución sistémica de BAFF en modelos múridos y en algunos pacientes con enfermedades autoinmunes apoyan el papel de este factor en autoinmunidad. El análisis de cohortes de pacientes muestran que BAFF está sistémicamente elevado en enfermedades como lupus eritematoso sistémico, artritis reumatoide, síndrome de Sjögren, y granulomatosis de Wegener. En algunos de estos pacientes se ha reportado también incrementos locales de BAFF en tejidos afectados como las glándulassalivales de pacientes con Sjögren, líquido sinovial en pacientes con artritisreumatoide, y lesiones de pacientes con esclerosis múltiple. Igualmente se hareportado aumento en los niveles séricos de BAFF en pacientes con esclerodermia. Todo esto sugiere un papel importante de esta molécula en la estimulación de las células (..) (AU)

B-cell activating factor of the tumor necrosis factor family (BAFF) isan important protein with regulatory functions in B-cell survival, maturation and differentiation, as well as the development of lymphoid organs through several receptors. BAFF’s features, mechanisms of signaling, and immune functions are being studied in murine models. Abnormal expression has been described in autoimmune, neoplastic and infectious diseases in both the mouse and the human being. The overproduction and the systemic distribution of BAFF (..) (AU)

The genetics of hypogammaglobulinemia

No disponible

No disponible