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1.
Allergol. immunopatol ; 47(5): 457-466, sept.-oct. 2019. tab, graf
Artigo em Inglês | IBECS | ID: ibc-186520

RESUMO

Background: Common variable immunodeficiency (CVID) is a heterogeneous group of primary antibody deficiencies defined by marked reductions in serum IgG, IgA and/or IgM levels and recurrent bacterial infections. Some patients are associated with defects in T cells and regulatory T cells (Tregs), resulting in recurrent viral infections and early-onset autoimmune disease. Methods: We analyzed whether there is an association between Tregs cells (CD4+CD25+CD127low and CD4+CD25+FoxP3+); memory T cells (CD4+CD45RO+); memory B cells (CD19+CD27-IgD-); and CD21low B cells (CD19+CD38lowCD21low); as well as autoimmune manifestations in 36 patients with CVID (25 women and 11 men, mean age 24 years), all by flow cytometry. Results: Fourteen patients presented with autoimmune diseases (AI) (39%), including 11 with autoimmune thrombocytopenia (ITP) (31%); two with vitiligo (6%); one with systemic lupus erythematosus (LES) (3%); and one with multiple sclerosis (MS) (3%). CVID patients with AI had a reduced proportion of Tregs (both CD4+CD25+CD127low and FoxP3+ cells) compared with healthy controls. CVID patients with AI had expanded CD21low B cell populations compared with patients who did not have AI. A correlation between increased CD4+CD45RO T cell populations and reduced Tregs was also observed. Conclusions: Our results showed that 39% of patients with CVID had AI and reduced Tregs populations. Research in this area might provide noteworthy data to better understand immune dysfunction and dysregulation related to CVID


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Assuntos
Humanos , Masculino , Feminino , Adulto Jovem , Doenças Autoimunes/metabolismo , Linfócitos B/imunologia , Imunodeficiência de Variável Comum/imunologia , Linfócitos T CD4-Positivos/imunologia , Linfócitos T Reguladores/imunologia , Subpopulações de Linfócitos B/imunologia , Citometria de Fluxo , Fatores de Transcrição Forkhead/metabolismo , Antígenos Comuns de Leucócito/metabolismo , Receptores de Complemento 3d/metabolismo
2.
Med. clín (Ed. impr.) ; 153(6): 225-231, sept. 2019. graf, tab
Artigo em Espanhol | IBECS | ID: ibc-184027

RESUMO

Fundamento y objetivo: Analizar la asociación entre concentraciones de interferón-1alpha (INF1alpha), interleucina 10 (IL-10) y BLyS con la actividad clínica en el lupus eritematoso sistémico (LES). Pacientes y métodos: Estudio observacional transversal de 142 pacientes con LES y 34 controles sanos mediante analítica de sangre y orina y revisión de la historia clínica. La concentración sérica de citocinas se determinó mediante métodos colorimétricos. El análisis bioestadístico se realizó con R (3.3.2). Resultados: El 69% de pacientes mostraron al menos una citocina aumentada. Las tres citocinas están más elevadas en pacientes que en controles (p<0,001, p=0,005 y p=0,043), siendo INF1alpha el más frecuente. Los pacientes fueron categorizados según las concentraciones de las tres citocinas. Encontramos una asociación significativa entre concentraciones elevadas de IL-10/INF1alpha y una mayor actividad clínica según SELENA-SLEDAI (p<0,0001) y, en menor medida, con concentraciones aumentadas de INF1alpha/IL-10/BLyS. Concentraciones elevadas de IL-10/INF1alpha e INF1alpha/IL-10/BLyS se relacionaron con un mayor consumo de C3-C4 (p<0,001 y p=0,001) y títulos elevados de anti-dsDNA (p=0,001 y p=0,002). Concentraciones elevadas de INF1alpha/BLyS se relacionaron con títulos más altos de anti-dsDNA (p=0,004) y positividad ENA (p<0,001). Concentraciones altas de INF1alpha/IL-10/BLyS se relacionaron con la positividad de ANA (p<0,001) y anticuerpos antifosfolípidos (p=0,004). Conclusiones: INF1alpha, IL-10 y BLyS están más elevados en pacientes con LES que en controles sanos. El aumento de IL-10, asociado o no a aumento de BLyS y/o INF1alpha, es la citocina que mejor se ajusta a la actividad clínica del LES medida con métodos clásicos


Background and objective: to analyse the association between interferon-1alpha (INF1alpha), interleukin-10 (IL-10) and BLyS concentrations and clinical activity in systemic lupus erythematosus (SLE). Patients and methods: A cross-sectional, observational study of 142 SLE patients and 34 healthy controls was performed, through a complete blood and urine test and review of their medical history. Serum concentration of INF1alpha, IL-10 and BLyS was determined by colorimetric methods. A biostatistical analysis was performed with R (3.3.2.). Results: 69% of our SLE patients showed at least one cytokine increased. INF1alpha, IL-10 and BLyS are higher in SLE patients than in healthy controls (P<.001, P=.005 and P=.043, respectively), being INF1alpha the most frequent. Patients were categorised according to low or high concentrations of the three cytokines. We found a significant association between increased IL-10/INF1alpha concentrations and a higher clinical activity measured by SELENA-SLEDAI (P<.0001) and, to a lesser extent, an association with increased INF1alpha/IL-10/BLyS concentrations. Elevated levels of IL-10/INF1alpha and INF1alpha/IL-10/BLyS related to increased C3-C4 consumption (P<.001 and P=.001 respectively) and anti-dsDNA titres (P=.001 and P=.002 respectively). Elevated INF1alpha/BLyS related to higher anti-dsDNA titres (P=.004) and ENA positivity (P<.001). Increased levels of INF1alpha/IL-10/BLyS related to positivity of ANAs (P<.001) and APL (P=.004). Conclusions: INF1alpha, IL-10 and BLyS are higher in SLE patients than in healthy controls. Increased IL-10 levels, regardless of whether or not there were also increased levels of BLyS and/or INF1alpha, was the cytokine that best fit with clinical activity in SLE measured with classic methods


Assuntos
Humanos , Feminino , Criança , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/sangue , Interferon Tipo I/sangue , Interleucina-10/sangue , Linfócitos B/metabolismo , Biomarcadores/sangue , Biomarcadores/urina , Estudos Transversais , Lúpus Eritematoso Sistêmico/urina , Colorimetria/métodos , Bioestatística , Anticorpos Antifosfolipídeos , Inquéritos e Questionários , Ensaio de Imunoadsorção Enzimática , Citocinas/sangue , Citocinas/urina
3.
Allergol. immunopatol ; 47(4): 372-327, jul.-ago. 2019. graf, tab
Artigo em Inglês | IBECS | ID: ibc-186509

RESUMO

Introduction: Chronic granulomatous disease (CGD) is a disorder of phagocyte function, characterized by pyogenic infections and granuloma formation caused by defects in NADPH oxidase complex activity. Although the effect of CGD mainly reflects the phagocytic compartment, B cell responses are also impaired in patients with CGD. Materials and methods: Flow cytometric analysis was performed on peripheral blood samples from 35 CGD patients age-matched with healthy controls (HC). The target cells of our study were the naive (IgD+/CD27-), memory (IgD-/CD27+), and B1a (CD5+) cells. Immunoglobulins (Igs) were also measured. This study was performed in a Latin American cohort. Results: We found significantly higher levels of naive B cells and B1a cells, but lower levels of memory B cells were found in CGD patients compared to HC. There was no significant difference of cell percentages per inheritance type. Discussion: Our findings suggest that the deficiency of NADPH oxidase components can affect the differentiation of naive B cells to memory B cells. Consequently, memory cells will be low, which also influenced the expression of CD27 in memory B cells and as a result, the percentage of naive cells increases. An altered phenotype of B lymphocytes in CGD patients may contribute to the opportunistic infections and autoimmune disorders that are seen in this disease


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Assuntos
Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Adolescente , Adulto Jovem , Adulto , Linfócitos B/imunologia , Subpopulações de Linfócitos B/imunologia , Doença Granulomatosa Crônica/imunologia , NADPH Oxidase 2/genética , Separação Celular , Estudos de Coortes , Citometria de Fluxo , Doença Granulomatosa Crônica/genética , Memória Imunológica , México , Membro 7 da Superfamília de Receptores de Fatores de Necrose Tumoral/metabolismo
4.
Allergol. immunopatol ; 47(3): 234-240, mayo-jun. 2019. graf, tab
Artigo em Inglês | IBECS | ID: ibc-186483

RESUMO

Introduction and objectives: Allergic rhinitis (AR) is a classic Th2-mediated disease, with important contributions to the pathology of interleukins 4, 5, and 13. The co-stimulatory molecule of OX40 and its ligand interaction participate in the immune response by regulation of Th1/Th2 cells balance. Considering the paucity of information on the relation between OX40 ligand (OX40L) and AR, this study aimed to examine its expression on B lymphocytes. Patients and methods: This case-control study consisted of 20 AR patients and 20 healthy subjects. The serum level of total immunoglobulin E (IgE) was measured using the electro-chemiluminescence (ECL) technology. The percentage of B-lymphocytes expressing OX40L was assessed by flow cytometry. The amounts of IL-4 in CD4+ T cells culture supernatant was also measured by the enzyme-linked immunosorbent assay (ELISA). Results: OX40L expression on B lymphocytes of patients was significantly higher than the control group (44.32 ± 19.21% vs. 2.79 ± 2.48% respectively, p < 0.001). In AR patients, OX40L expression correlated positively with the levels of serum total IgE and IL-4 produced by CD4+ T lymphocytes (p < 0.01 - p < 0.05) respectively. Conclusions: Collectively, the findings of this work suggest that there is a relationship between the OX40L expression level on B lymphocytes and allergic markers such as IgE and IL-4 in patients with allergic rhinitis


No disponible


Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Linfócitos B/imunologia , Biomarcadores/sangue , Imunoglobulina E/sangue , Interleucina-4/sangue , Ligante OX40/metabolismo , Rinite Alérgica/imunologia , Células Th2/imunologia , Antígenos CD4/metabolismo , Estudos de Casos e Controles , Regulação para Cima , Células Cultivadas
5.
Allergol. immunopatol ; 47(1): 52-59, ene.-feb. 2019. tab, graf
Artigo em Inglês | IBECS | ID: ibc-180772

RESUMO

Background: Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by low serum levels of immunoglobulins (Igs) and recurrent infection. In most CVID patients, a defect in the differentiation of B cells into plasma cells has been observed. Several factors play an important role in the proliferation and differentiation of B cells, including IRF4 and XBP1 transcription factors. Methods: In the present study we investigated the expression of IRF4 and XBP1 in the B-cells of CVID and healthy controls (HCs). For this purpose, we assessed the expression of IRF4 and XBP1 at both mRNA and protein levels by real time-PCR and flow cytometry, respectively. Results: We found that IRF4 expression was significantly increased in CVID patients compared with controls. Although the XBP1 protein level was lower in patients in comparison to controls, this difference was not significant. Conclusion: Taken together, increased IRF4 expression could be involved in defective functions of B cells in CVID patients


No disponible


Assuntos
Humanos , Masculino , Pré-Escolar , Criança , Adolescente , Adulto Jovem , Linfócitos B/imunologia , Imunodeficiência de Variável Comum/imunologia , Fatores Reguladores de Interferon/metabolismo , Citometria de Fluxo , Fatores Reguladores de Interferon/genética , RNA Mensageiro/genética , Regulação para Cima , Proteína 1 de Ligação a X-Box/genética , Proteína 1 de Ligação a X-Box/metabolismo
7.
Rev. esp. patol ; 50(4): 262-267, oct.-dic. 2017. ilus
Artigo em Espanhol | IBECS | ID: ibc-166045

RESUMO

El linfoma intravascular es un tipo de linfoma extraganglionar, generalmente de células B, definido por una proliferación de linfocitos atípicos dentro de la luz de vasos de pequeño y mediano calibre. Desde su descripción en 1959 ha recibido diferentes denominaciones. En la actualidad, la Organización Mundial de la Salud se refiere a esta entidad como linfoma intravascular de células B grandes. Presentamos un caso caracterizado por deterioro neurológico de evolución rápida y progresiva consecutivo a eventos isquémicos multifocales y recurrentes de etiología inicialmente indeterminada. En el estudio post mortem limitado a la cavidad craneal se detectó una proliferación celular atípica en la luz de vasos de pequeño y mediano calibre. Con técnicas inmunohistoquímicas se confirmó el origen linfoide de las células neoplásicas intravasculares y se estableció el diagnóstico de linfoma intravascular de células B grandes (AU)


Intravascular lymphoma is a type of extranodal lymphoma, usually composed of B-cells, resulting from a proliferation of atypical lymphocytes within the lumina of small to medium sized vessels. Since its initial description in 1959, it has had many names but currently the World Health Organization refers to this entity as intravascular large B-cell lymphoma. We present a case which presented with rapid progressive neurological deterioration and consecutive progressive multifocal and recurrent ischemic events of unknown origin. The postmortem study of the cranial cavity revealed atypical cell proliferation within the lumina of small to medium sized vessels. The lymphoid origin of intravascular tumor cells was confirmed by immunohistochemistry, establishing a diagnosis of intravascular large B-cell lymphoma (AU)


Assuntos
Humanos , Feminino , Idoso , Linfócitos B/patologia , Linfoma Extranodal de Células T-NK/patologia , Acidente Vascular Cerebral/complicações , Neoplasias Neuroepiteliomatosas/patologia , Imuno-Histoquímica/métodos , Diagnóstico Diferencial , Imunoterapia/métodos , Rituximab/uso terapêutico , Ciclofosfamida/uso terapêutico , Vincristina/uso terapêutico , Prednisona/uso terapêutico
8.
Allergol. immunopatol ; 45(5): 439-444, sept.-oct. 2017. tab
Artigo em Inglês | IBECS | ID: ibc-166997

RESUMO

Background: The purpose of this study is to examine the changes in B lymphocyte subsets in patients receiving allergen immunotherapy. Methods: B lymphocyte subsets of patients before immunotherapy and one year after immunotherapy began were examined using the flow cytometric method. Age-matched healthy children served as the control group. Results: Twenty-two patients with asthma and/or allergic rhinitis and 14 healthy, age-matched controls were included in the study. The median age of the patients was 13 years old (range: 6-20 years), and eleven (50.0%) were male. The median age of the healthy controls was also 13 years old (range: 7-17), and seven (50.0%) were male. In the age group from 11 to 15 years; the patients’ relative and absolute counts of active and mature sensitive B cells were higher than those of the healthy children (p = 0.027-0.012 and p = 0.032-0.010, respectively) before immunotherapy. The relative and absolute counts of active B cells before immunotherapy were also significantly higher than those of after immunotherapy (p = 0.001-0.001, p = 0.025-0.037, and p = 0.029-0.035, respectively). Before immunotherapy, the relative and absolute counts of mature sensitive B cells were significantly higher than those obtained after immunotherapy (p = 0.024-0.006) in the 11–15-year-old age group. Conclusions: Allergen immunotherapy directly influences B cell differentiation and causes a decrease in the count of active B cells. This finding is relevant because the B cell count can be used as a guide in the assessment of an individual patient's treatment response and also when determining whether to continue the immunotherapy (AU)


No disponible


Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Adulto Jovem , Hipersensibilidade/terapia , Imunoterapia Ativa/métodos , Linfócitos B/imunologia , Estudos de Casos e Controles , Receptores de IgE/análise , Autoimunidade/imunologia , Asma/terapia , Rinite Alérgica/terapia
9.
Allergol. immunopatol ; 45(3): 290-296, mayo-jun. 2017. tab, ilus
Artigo em Inglês | IBECS | ID: ibc-162393

RESUMO

Background. Neonatal jaundice is one of the most common problems that affect newborn infants, and phototherapy is usually used for treatment. Objectives. Evaluation of the effect of phototherapy on neonatal immune system through measuring the percentage of B and T lymphocytes and determining the frequency of development of infections and need for hospitalisation during the first six months of life. Methods. A prospective cohort study was conducted on 50 full term new-borns; 25 with indirect hyperbilirubinaemia and treated with conventional phototherapy and 25 healthy matched neonates as untreated controls. The percentages of CD19+, CD4+ and CD8+ lymphocytes were measured by flow cytometry before phototherapy and 72h after exposure. Follow-up of the study group for the occurrence of infections for a period of six months after phototherapy. Results. The study showed a significant difference in CD19+ lymphocytes percentage between patients before phototherapy and controls (P value<0.01), also a significant correlation between serum levels of total bilirubin in patients and CD19+ lymphocytes percentage (P value<0.05). There was no significant difference between the percentages of CD19+, CD4+ and CD8+ lymphocytes in patients before or after 72h of exposure to phototherapy (P value>0.05). Also, there was no correlation between the percentages of CD19+, CD4+ and CD8+ lymphocytes after 72h of exposure to phototherapy and the occurrence of infections (Gastrointestinal tract and Respiratory tract infection) after six months of follow-up (P value>0.05). More studies are needed with larger number of patients to determine the effect of phototherapy on immune system (AU)


No disponible


Assuntos
Humanos , Masculino , Feminino , Lactente , Linfócitos B/efeitos da radiação , Linfócitos T/efeitos da radiação , Fototerapia , Sistema Imunitário/efeitos da radiação , Hiperbilirrubinemia/complicações , Estudos Prospectivos , Estudos de Coortes , Antígenos CD19/análise , Antígenos CD4/análise , Antígenos CD8/análise , Citometria por Imagem
11.
Acta pediatr. esp ; 75(1/2): e11-e13, ene.-feb. 2017. ilus
Artigo em Espanhol | IBECS | ID: ibc-160198

RESUMO

Introducción: El linfoma linfoblástico de precursores de células B (LLB) es responsable del 2% de los casos de los linfomas diagnosticados en la edad pediátrica. El retraso en su diagnóstico es habitual, debido a la poca especificidad de los síntomas asociados. Exponemos un caso clínico con una presentación clínica muy poco común. Caso clínico: Paciente de 14 años de edad que acudió a nuestra consulta por un dolor en la rodilla, la cadera y el muslo izquierdo de 40 días de evolución, que le provocó incapacidad para deambular 1 semana antes de su ingreso. En su abordaje inicial se indicó tratamiento con ketorolaco y piroxicam, lo que propició una mejoría transitoria del dolor. Se realizaron radiografías de la columna lumbar, la cadera y la extremidad inferior, una tomografía computarizada (TC) y una resonancia magnética (RM). Las radiografías detectaron lesiones líticas, que posteriormente fueron documentadas en la TC, en el trocánter y la cabeza del fémur izquierdo y en la primera vértebra lumbar. La RM demostró, además, la presencia de adenomegalias supraclaviculares y una masa paravertebral en la octava vértebra torácica. El aspirado de médula ósea fue negativo para la infiltración, y la biopsia de una de las adenomegalias supraclaviculares reveló un linfoma tipo B. Conclusión: El LLB es una variedad de linfoma unicelular poco común en pediatría, que generalmente se diagnostica en estadios avanzados por su rápido crecimiento (AU)


Introduction: B-cell lymphoblastic lymphoma contributes 2% of the diagnosed lymphomas in the pediatric age. The diagnosis is often delayed by the low specificity of presenting symptoms. We illustrate an uncommon B-cell lymphoblastic lymphoma presentation. Case report: A 14-year old female presented with a 40-day history of left knee, hip and thigh pain that produced intermittent limping, associated with one week history of limping. During her initial medical assistance ketorolac and piroxicam were prescribed, partially decreasing pain. She evolved walking disability, leading her to hospitalization were X-rays, CT scan and MRI were ordered. Osteolithic lesions were found and confirmed by CT scan in 1st lumbar vertebra and left femur head and trochanter. MRI found bilateral supraclavicular lymphadenopathies along a paravertebral tumor at T8 level. Marrow aspiration was negative and biopsy of the supraclavicular masses revealed a B-cell lymphoma, which was further characterized by immunohistochemistry in B-cell lymphoblastic lymphoma. Conclusion: B-cell lymphoblastic lymphoma is a rare tumor in children and is usually diagnosed in advanced stages due to their rapid growth and delay in the diagnosis. The most important diagnostic tool for the general pediatrician is to maintain clinical suspicion due to the low specificity of the clinical symptoms (AU)


Assuntos
Humanos , Masculino , Adolescente , Linfoma não Hodgkin/complicações , Linfoma não Hodgkin , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Leucemia-Linfoma Linfoblástico de Células Precursoras , Traumatismos da Coluna Vertebral/complicações , Punção Espinal , Fraturas da Coluna Vertebral/complicações , Linfócitos B/citologia , Linfócitos B/patologia , Linfócitos B , Espectroscopia de Ressonância Magnética/métodos , Biometria/instrumentação
12.
J. investig. allergol. clin. immunol ; 27(1): 46-57, 2017. tab, graf
Artigo em Inglês | IBECS | ID: ibc-160497

RESUMO

Background and Objectives: Allergic asthma and rhinitis are common in pregnancy. The immune mechanisms underlying the effects of asthma on pregnancy and vice versa are not completely understood. The aim of this study was to investigate changes in regulatory T and B cells in asthmatic women from late pregnancy to postpartum. Methods: Four groups of women were enrolled for this study: asthmatic (n=23) and healthy (n=43) third trimester-pregnant women and asthmatic (n=33) and healthy (n=35) nonpregnant women. Pregnant women were also evaluated postpartum (>6 weeks after delivery). Blood samples were taken from each woman and flow cytometry was used to characterize circulating regulatory T cells (Tregs) and regulatory B cells (Bregs). Foxp3 expression was assessed in CD4DimCD25Hi Tregs. Results: Tregs did not vary significantly from pregnancy to postpartum in asthmatic or healthy women, but CD24HiCD38Hi Bregs decreased in pregnancy and increased significantly postpartum. Foxp3 expression in Tregs was also impaired during pregnancy in both asthmatic and healthy women, but recovered postpartum. Asthmatic pregnant women had higher Foxp3 expression levels than healthy pregnant women (P=.007), probably due to the use of control medication. Conclusions: Women with controlled asthma showed variations in regulatory cell subsets during pregnancy and postpartum. A similar pattern was observed for Foxp3 expression and CD24HiCD38Hi Bregs during this period, corroborating the interaction between Tregs and Bregs in immune responses. Considering the immunomodulatory potential of these immune mediators, more studies are needed to evaluate their relationship with asthma and rhinitis complications in pregnancy (AU)


Introducción y Objetivos: El asma y la rinitis alérgica son enfermedades comunes durante el embarazo. A pesar de ello, no están completamente esclarecidos los mecanismos inmunológicos del embarazo implicados en el asma y viceversa. Este trabajo tuvo como objetivo el estudiar la evolución de los linfocitos T y B reguladores en mujeres asmáticas embarazadas, desde fases tardías del embarazo hasta después del parto. Métodos: Se incluyeron cuatro grupos de mujeres para este estudio: mujeres embarazadas en su tercer trimestre, asmáticas (n = 24) y sanas (n = 43), y mujeres no embarazadas, asmáticas (n = 33) y sanas (n = 35). Las mujeres embarazadas también fueron evaluadas después del parto (> 6 semanas después del parto). Se tomaron muestras de sangre de cada mujer y se realizó citometría de flujo para caracterizar los linfocitos T y B reguladores circulantes. La expresión de Foxp3 se evaluó en los linfocitos T reguladores CD4DimCD25Hi. Resultados: En las mujeres embarazadas, tanto sanas como asmáticas, los linfocitos T reguladores no oscilaron de manera significativa desde el embarazo hasta después del parto. Sin embargo, en los linfocitos B reguladores CD24HiCD38Hi, se observó una disminución durante el embarazo que aumentó significativamente después del parto. La expresión de Foxp3 en los linfocitos T reguladores también se vio alterada durante el embarazo tanto en las mujeres embarazadas asmáticas como en las sanas, normalizándose en el posparto. No obstante, las mujeres asmáticas embarazadas presentaron niveles de expresión de Foxp3 superiores a los de las mujeres embarazadas sanas (p = 0,007), probablemente debido a la utilización de medicación de control. Conclusiones: Las mujeres con asma controlada, durante el embarazo y después del parto, presentan variaciones en los diferentes subtipos linfocitos reguladores. El similar comportamiento que se observa para la expresión de Foxp3 y los linfocitos B reguladores CD24HiCD38Hi apoya la interacción que se establece en la respuesta inmunitaria, entre los linfocitos T y B reguladores, durante este período. Teniendo en cuenta el potencial inmunomodulador de estos mecanismos, se necesitan más estudios para evaluar su relación con las complicaciones del asma y la rinitis durante el embarazo (AU)


Assuntos
Humanos , Feminino , Gravidez , Adulto , Linfócitos B/imunologia , Linfócitos T/imunologia , Asma/complicações , Asma/imunologia , Complicações na Gravidez/imunologia , Antígenos CD4/imunologia , Subunidade alfa de Receptor de Interleucina-2/imunologia , Linfócitos T Reguladores/imunologia , Citometria de Fluxo/métodos , Antropometria/métodos , Análise de Dados/métodos
13.
Pediatr. aten. prim ; 18(71): e111-e114, jul.-sept. 2016.
Artigo em Espanhol | IBECS | ID: ibc-156613

RESUMO

La enfermedad de Bruton o agammaglobulinemia ligada al cromosoma X es una inmunodeficiencia primaria que cursa con la disminución drástica o la inexistencia de inmunoglobulinas en la sangre periférica. Esto va a originar una predisposición a desarrollar infecciones bacterianas recurrentes en el periodo de lactancia. Lo más importante y complejo a su vez es establecer el diagnóstico de sospecha, ya que se trata de una enfermedad infrecuente que cursa con manifestaciones muy comunes, como son las infecciones. Una vez sospechada la enfermedad, es importante derivar al paciente a un centro especializado para realizar los análisis pertinentes; en primer lugar, un análisis de sangre con recuento de inmunoglobulinas, y si están descendidas hay que solicitar la determinación de subpoblaciones linfocitarias mediante citometría de flujo. El diagnóstico de certeza se establece mediante análisis genéticos. El tratamiento consiste en la administración temprana de gammaglobulina intravenosa. Presentamos el caso de un paciente en el que una buena historia clínica permitió un diagnóstico precoz de enfermedad de Bruton, previo a la aparición de potenciales complicaciones (AU)


Bruton’s disease or X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by severe hypogammaglobulinemia. This causes increased susceptibility to bacterial recurrent infections at young age. It is very important, but difficult, to establish a diagnosis based on suspicion because of the common clinical symptoms of this rare disease. Once XLA is suspected, it is very important to refer the patient to a specialized hospital for specific analysis to confirm the disease. First, a blood analysis is done to see if antibody levels are low. In that case, a fluorocytometric analysis is needed to study the antibody classes. Genetic analysis shows mutation of BTK gene resulting in defective B cell differentiation and it must be done for diagnosis certainty. Treatment is based on intravenous immunoglobulin administration for life. We report a case in which a good clinical history was the key for an early diagnosis and treatment of a patient with Bruton´s disease before any potential complications appeared (AU)


Assuntos
Humanos , Masculino , Lactente , Agamaglobulinemia/complicações , Agamaglobulinemia/diagnóstico , Emigrantes e Imigrantes/estatística & dados numéricos , Citometria de Fluxo/instrumentação , Citometria de Fluxo , gama-Globulinas/uso terapêutico , Antibacterianos/uso terapêutico , Síndromes de Imunodeficiência/complicações , Linfócitos B , Diagnóstico Precoce , Atenção Primária à Saúde/métodos , Atenção Primária à Saúde/tendências , Comorbidade , Imunoglobulina G/administração & dosagem , Imunoglobulina G/análise
14.
Reumatol. clín. (Barc.) ; 12(1): 39-46, ene.-feb. 2016. tab
Artigo em Espanhol | IBECS | ID: ibc-149358

RESUMO

Las vasculitis asociadas a ANCA son enfermedades autoinmunes crónicas que se caracterizan por inflamación y destrucción de vasos de pequeño tamaño. El rituximab es un tratamiento efectivo para la fase de inducción de estas patologías. Durante los últimos años, varios estudios no controlados han reportado que también es eficaz durante la fase de mantenimiento terapéutico. En estas series, el fármaco se administró solo durante las recaídas, a intervalos fijos o sobre la base en cambios en algunos biomarcadores. Los resultados del estudio MAINRITSAN mostraron que el rituximab es superior a la azatioprina como terapia de mantenimiento en estas enfermedades. Este trabajo de revisión resume la información más reciente sobre el uso de rituximab como opción para la fase de mantenimiento de las vasculitis asociadas a ANCA, detallando su efectividad, los diversos protocolos de administración, el perfil de seguridad y el uso potencial de biomarcadores para guiar el tratamiento (AU)


ANCA-associated vasculitides (AAV) are chronic autoimmune diseases characterized by inflammation and destruction of small vessels. Rituximab is now licensed for use as a remission-induction agent in the treatment of these disorders. During recent years, several non-controlled studies have suggested that rituximab may be of value in maintaining disease remission in AAV. In these series, 3 techniques have been tried: 'watch-and-wait', repeated cycles in fixed intervals, or administration based on proposed biomarkers. More importantly, the results of the MAINRITSAN trial showed that this anti-CD20 agent is superior to azathioprine for preventing major relapses in AAV. This review summarizes current information regarding the effectiveness, timing, dosing, duration and safety of rituximab as a valid option for remission maintenance (AU)


Assuntos
Humanos , Masculino , Feminino , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/sangue , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Preparações Farmacêuticas/administração & dosagem , Terapêutica/métodos , Poliangiite Microscópica/genética , Doenças Cardiovasculares/diagnóstico , Linfócitos B/citologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/metabolismo , Preparações Farmacêuticas/metabolismo , Terapêutica/instrumentação , Poliangiite Microscópica/metabolismo , Doenças Cardiovasculares/complicações , Estudo Observacional , Linfócitos B/patologia
15.
J. investig. allergol. clin. immunol ; 26(4): 233-243, 2016. tab
Artigo em Inglês | IBECS | ID: ibc-154935

RESUMO

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 (AU)


La 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 (AU)


Assuntos
Humanos , Masculino , Feminino , Linfócitos T/imunologia , Imunodeficiência de Variável Comum/epidemiologia , Imunodeficiência de Variável Comum/imunologia , Imunodeficiência de Variável Comum/prevenção & controle , Linfócitos B/imunologia , Plasmócitos/imunologia , Linfócitos T Reguladores/imunologia , Imunodeficiência de Variável Comum/fisiopatologia , Apoptose/imunologia , Vírus Auxiliares/imunologia , Linfócitos T Reguladores
16.
Clin. transl. oncol. (Print) ; 17(11): 841-846, nov. 2015. tab
Artigo em Inglês | IBECS | ID: ibc-143453

RESUMO

MicroRNA (miRNA), a class of non-proteincoding RNAs, plays a critical role in many cellular processes, such as invasion, proliferation and migration, and also function in disease pathology. The transcription factor and proto-oncogene B cell CLL/lymphoma 6 (BCL6) is aberrantly expressed in various cancers. An increasing body of evidence has demonstrated that miRNAs and BCL6 can target one another and mutually adjust their expression which are of great importance in the pathogenesis of various cancers. In this report, we summarize the mutual interaction between miRNAs and BCL6, which have been studied in cancers, highlighting their mechanisms and potential therapeutic targets in cancers (AU)


No disponible


Assuntos
Feminino , Humanos , Masculino , MicroRNAs , Neoplasias/diagnóstico , Linfócitos B/patologia , Proteínas Proto-Oncogênicas c-bcl-6/administração & dosagem , Proteínas Proto-Oncogênicas c-bcl-6/análise , Proteínas Proto-Oncogênicas c-bcl-6 , Neoplasias da Mama/diagnóstico , Linfoma não Hodgkin/diagnóstico , Carcinoma Hepatocelular/diagnóstico , Proto-Oncogenes/fisiologia , Proteínas Proto-Oncogênicas , Proteínas Oncogênicas , Linfoma Difuso de Grandes Células B/diagnóstico
17.
Allergol. immunopatol ; 43(5): 477-481, sept.-oct. 2015. tab
Artigo em Inglês | IBECS | ID: ibc-141109

RESUMO

Background: Ataxia telangiectasia (A-T) is a genetic disorder caused by the homozygous mutation of the A-T mutated gene. It is frequently associated with variable degrees of cellular and humoral immunodeficiency. However, the immune defects in A-T patients are not well characterized. To the best of our knowledge, no studies have focused on the major lymphocyte subpopulations and recent thymic emigrants of A-T patients in comparison with age-matched healthy controls. Methods: Following the European Society for Immunodeficiencies criteria, 17 patients diagnosed with A-The, and 12 age-matched healthy children were assigned to the study. Both patients and healthy controls were grouped as 1–5, 6–10, 11–15, and 15+ years. By using a flow cytometer, major lymphocyte subpopulations and CD4+CD45RA+CD31+ recent thymic emigrants were determined as percentage and absolute cell numbers and compared. Results: No significant differences in all lymphocyte subpopulations were observed between the age groups of A-T patients. Compared to the healthy controls, there was a decrease in T cells, effector memory T4 cells, B cells, naïve B cells, naïve T4 cells, switched B cells, and recent thymic emigrants and an increase in active T8 cells and non-switched B cells in the percentage and absolute number of some cell populations in the A-T group. Conclusions: This study showed that effector functions in some cell lymphocyte populations were decreased in A-T patients (AU)


No disponible


Assuntos
Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Ataxia Telangiectasia/imunologia , Subpopulações de Linfócitos , Linfócitos T , Linfócitos T CD4-Positivos , Linfócitos T CD8-Positivos , Linfócitos B , Linfopenia/epidemiologia , Citometria de Fluxo , Imunidade Humoral , Síndromes de Imunodeficiência , Antígenos CD4 , Antígenos Comuns de Leucócito , Molécula-1 de Adesão Celular Endotelial a Plaquetas
18.
Rev. esp. patol ; 48(3): 159-162, jul.-sept. 2015. ilus
Artigo em Espanhol | IBECS | ID: ibc-139259

RESUMO

La inmunodeficiencia variable común es una inmunodeficiencia primaria por hipogamaglobulinemia de IgG e IgA poco frecuente en la población. Clásicamente se presenta durante la juventud y se diagnostica durante la investigación de infecciones respiratorias y gastrointestinales a repetición. Estos pacientes pueden además presentar enfermedades autoinmunes, inflamatorias, neoplásicas, malabsorción y problemas granulomatosos no infecciosos, que afectan el pulmón, ganglios, bazo, hígado, y menos usualmente la piel. Presentamos el caso de una paciente diagnosticada de inmunodeficiencia variable común que se presenta en la consulta dermatológica con múltiples nódulos eritemato-costrosos en miembros, donde se hallaron únicamente granulomas asépticos (AU)


Common variable immunodeficiency is a rare, primary immunodeficiency characterized by hypogammaglobulinemia of IgG and IgA. It classically presents as recurrent respiratory and gastrointestinal infections in young patients, who may also have autoimmune and inflammatory disease, malignancies, malabsorption and non-infectious granulomas, mainly located in the lung, lymph nodes, spleen, liver and, less frequently, in the skin. We report the case of a patient diagnosed with common variable immunodeficiency who presented in the dermatology clinic with multiple, erythematous, crusted nodules on the limbs. Only aseptic granulomas were found (AU)


Assuntos
Feminino , Humanos , Granuloma/patologia , Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/diagnóstico , Imunodeficiência de Variável Comum/patologia , Imunoglobulina A/isolamento & purificação , Infecções Respiratórias/patologia , Diagnóstico Diferencial , Antígenos CD4 , Síndromes de Imunodeficiência/patologia , Imunoglobulina G , Linfócitos B/patologia , Tomografia Computadorizada de Emissão , Antígenos CD8
19.
Med. clín (Ed. impr.) ; 145(5): 206-210, sept. 2015. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-139671

RESUMO

El lupus eritematoso sistémico es una enfermedad autoinmune que está asociada con la producción anormal de autoanticuerpos por parte de linfocitos B autorreactivos. El estudio de las características fenotípicas del linfocito B y la identificación de receptores en su superficie, como BAFF-R, TACI y BCMA, como responsables de su supervivencia y maduración han contribuido en los últimos años al desarrollo de nuevas estrategias terapéuticas (AU)


Systemic lupus erythematosus is an autoimmune disease associated with an aberrant production of autoantibodies by self-reactive B lymphocytes. The study of the phenotypic characteristics of B lymphocytes and the identification of their surface receptors such as BAFF-R, TACI and BCMA, which are responsible of their survival and maturation, have contributed to the development of new therapeutic strategies in recent years (AU)


Assuntos
Feminino , Humanos , Masculino , Linfócitos B , Sobrevivência/fisiologia , Lúpus Eritematoso Sistêmico/terapia , Receptor do Fator Ativador de Células B/metabolismo , Receptor do Fator Ativador de Células B/uso terapêutico , Receptor do Fator Ativador de Células B/farmacocinética , Sistemas de Liberação de Medicamentos , Adjuvantes Farmacêuticos/uso terapêutico
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