Linfocitos B instruidos por bacterias potencian la capacidad citotóxica de linfocitos T CD8 naïve / [B lymphocytes instructed by bacteria enhance the cytotoxic capacity of naïve CD8 T lymphocytes]

Los linfocitos B desempeñan un papel bien conocido en la respuesta inmunitaria adaptativa, tanto a nivel humoral mediante la producción de anticuerpos y citoquinas como durante la activación de los linfocitos T CD4. Sin embargo, su capacidad para capturar, procesar y activar linfocitos T CD8 naïve mediante presentación cruzada (“cross-presentation”) es un campo en que los mecanismos moleculares que regulan dicha capacidad no están definidos. De forma previa, se sabía que las células B son capaces de incrementar las respuestas citotóxicas de linfocitos T CD8 durante la infección. En este trabajo recientemente publicado en EMBO Reports con Raquel García-Ferreras como primera autora, del laboratorio del Dr. Esteban Veiga en el Centro Nacional de biotecnología (CNB, Madrid), se demuestra cómo los linfocitos B capturan bacterias vía endocitosis a través de contactos con células dendríticas infectadas, siendo la transfagocitosis pues la vía preferida. Estos linfocitos B así instruidos procesan los componentes bacterianos mediante autofagia y presentan los antígenos producidos a linfocitos T CD8, que van a mejorar su capacidad citotóxica y por tanto su actividad para eliminar células diana. Para entender los mecanismos moleculares que regulan esto procesos los autores han utilizado diferentes modelos de ratón y cepas bacterianas derivadas de L monocytogenes, determinando mediante citometría de flujo y microscopía confocal la capacidad de presentación antigénica de los linfocitos B y la proliferación de los linfocitos T CD8. La transfagocitosis de dichas bacterias conlleva el uso de autofagia y esto ocurre mediante el aumento en la expresión de moléculas co-estimuladoras y del MHC-I y es dependiente de la autofagia no clásica. Las células CD8 citotóxicas entrenadas por los linfocitos B son así más efectivas en el reconocimiento de células tumorales que expresan antígenos específicos. ... (AU)

An atypical case of refractory passenger lymphocyte syndrome after renal transplantation / Un caso atípico de síndrome de linfocitos pasajeros refractarios después del trasplante renal

Background Passenger lymphocyte syndrome (PLS) causes immune-mediated hemolysis in solid and bone marrow transplant recipients. Donor-derived antibodies against the recipient erythrocyte drive the pathogenesis. It is a rare entity in kidney transplantation, and most of the cases are self-limited. Case presentation A 36-year-old woman presented with fatigue 13 days after living donor renal transplantation. The operation was uneventful, and she was discharged with normal graft functions on the 11th day of transplantation Findings were consistent with cold agglutinin disease at her admission. However, the cold agglutinin test was negative. Eventually, she was diagnosed with PLS. Refractory intravascular hemolysis and frank hemoglobinuria were also present in the patient. Hemolysis was resistant to steroids, intravenous immunoglobulin (IVIG), and Rituximab. Because of life-threatening anemia related to refractory PLS, mycophenolate and tacrolimus were interrupted. However, hemolysis persisted. Following that, immunoadsorption (IA) treatment was obtained. Unfortunately, graft loss occurred due to rejection despite the resolution of PLS after IA. Conclusion PLS is a rare and usually self-limited entity. Our case was an atypical refractory PLS that resembled cold agglutinin disease. Also, frank hemoglobinuria was observed related to severe intravascular hemolysis. These features have not been described before in PLS, to the best of our knowledge. Additionally, IA treatment had never been reported in the literature for PLS, as far as we know. Treatment and management could be a challenge in refractory PLS. Rituximab, IVIG, and extracorporeal treatments could be beneficial. It should be borne in mind that refractory PLS can cause graft and patient loss (AU)

Antecedentes El síndrome de linfocitos pasajeros (PLS) causa hemólisis inmunomediada en receptores de trasplantes sólidos y de médula ósea. Los anticuerpos derivados del donante contra el eritrocito receptor impulsan la patogénesis. Es una entidad rara en el trasplante de riñón y la mayoría de los casos son autolimitados. Presentación del caso Una mujer de 36 años presentó fatiga 13 días después del trasplante renal de donante vivo. La operación transcurrió sin incidentes y fue dada de alta con las funciones normales del injerto el día 11 del trasplante. Los hallazgos coincidían con la enfermedad por crioaglutininas en el momento de su ingreso. Sin embargo, la prueba de crioaglutininas fue negativa. Finalmente, le diagnosticaron PLS. La paciente también presentó hemólisis intravascular refractaria y hemoglobinuria franca. La hemólisis fue resistente a los esteroides, la inmunoglobulina intravenosa (IgIV) y el rituximab. Debido a la anemia potencialmente mortal relacionada con PLS refractario, se interrumpieron el micofenolato y el tacrolimus. Sin embargo, persistió la hemólisis. A continuación, se obtuvo el tratamiento de inmunoadsorción (IA). Desafortunadamente, la pérdida del injerto ocurrió debido al rechazo a pesar de la resolución de PLS después de la IA. Conclusión El PLS es una entidad rara y generalmente autolimitada. Nuestro caso fue un PLS refractario atípico que se asemejaba a la enfermedad por crioaglutininas. Además, se observó hemoglobinuria franca relacionada con hemólisis intravascular grave. Estas características no se han descrito antes en PLS, según nuestro leal saber y entender. Además, el tratamiento IA nunca se había informado en la literatura para PLS, hasta donde sabemos. El tratamiento y el manejo podrían ser un desafío en PLS refractarios. El rituximab, la IgIV y los tratamientos extracorpóreos podrían ser beneficiosos. Debe tenerse en cuenta que los PLS refractarios pueden provocar la pérdida del injerto y del paciente (AU)

Application of the diagnostic criteria for Common Variable Immunodeficiency in resource-limited settings

Introduction: Common variable immunodeficiency (CVID) is the most prevalent symptom-atic humoral deficiency; however, its heterogeneous presentation makes the diagnosis diffi-cult. The present study is aimed to verify the CVID diagnostic criteria as established by the European Society for Immunodeficiencies in 42 CVID patients from our outpatient clinic. Methods: Information was collected from their medical records and when needed, lymphocyte subpopulations in peripheral blood (PB) were performed by flow cytometry. Results: All the patients fulfilled the clinical working definition for CVID and showed decreased serum IgG and IgA at diagnosis. Over two-thirds of the patients had decreased memory B cell percentages. However, the remaining patients exhibited other quantitative B cell defects in PB. Evaluation of vaccination responses was only found in 13 records and 69% were not respon-sive. None of the patients were subjected to vaccination studies to both, T-cell dependent and independent antigens. The two required tests to evaluate T cell responses were performed in 84.2% of the patients and reported normal. Without the support of third-party payers, only 34.2% of our patients would have completed the required evaluations. Conclusions: Further efforts are needed to speed up CVID diagnosis in low-resourced settings, increasing the availability of the required resources and optimizing the healthcare supply chain (AU)

Estudio observacional retrospectivo de persistencia de infección por SARS-CoV-2 en pacientes tratados previamente con rituximab / Retrospective observational study of the persistence of SARS-CoV-2 infection in patients previously treated with rituximab

Introducción. La inmunodepresión inducida por rituximabpodría ser un factor de riesgo de mortalidad por COVID-19. Elobjetivo del estudio fue describir la prevalencia de infecciónpor SARS-CoV-2 en pacientes que habían recibido rituximab yconocer si conduce a una mayor persistencia del virus.Material y métodos. Estudio observacional retrospectivo depacientes que recibieron rituximab en los 6 meses previos al inicio dela pandemia, analizándose la presencia de infección. Se recogieronlas siguientes variables: edad, sexo, enfermedades previas, factoresde riesgo para COVID-19, dosis recibidas de rituximab, resultados delos test diagnósticos, hospitalización, tipo de soporte ventilatorio,desarrollo de eventos tromboembólicos y tratamiento recibido. Serealizó un análisis descriptivo de todas las variables y se compararonpacientes que se habían infectado (C+) y los que no (C-).Resultados. 68 pacientes habían recibido rituximab (mediana de dosis acumulada: 4.161mg (2.611–8.187,5)), 54,4%hombres con edad media de 60,8 años (15,7; 25-87). Se confirmó C+ en 22 pacientes, entre los cuales existían los siguientes antecedentes: 45,5% hipertensión arterial, 36,4% DiabetesMellitus, 31,8% tabaquismo/exfumador, 22,7% neumopatía,13,6% cardiopatía y 4,5% obesidad. No se apreciaron diferencias estadísticamente significativas entre C+ y C-. Sólo 2 pacientes C+ desarrollaron inmunidad y 10 de ellos (45,5%) nonegativizaron PCR a la finalización del seguimiento. No se encontró asociación con la dosis acumulada de rituximab. La tasade mortalidad en la C+ fue de 22,7%.Conclusiones. En nuestros pacientes tratados con rituximab y con infección por SARS-CoV2 se observó una peorevolución y una mayor persistencia de la infección, por lo quedebería valorarse el uso de otras alternativas durante la pandemia, ya que la disminución de la función de células B podríaproducir un mayor riesgo de evolución fatal por COVID-19. (AU)

Introduction. Rituximab-induced immunosuppressioncould be a risk factor for mortality from COVID-19. The aimof the study was to describe the prevalence of SARS-CoV-2infection in patients who have received rituximab and its association with a persistent viral infectionMaterial and methods. Retrospective observationalstudy of patients who received rituximab in the 6 monthsbefore to the onset of the pandemic. We analyzed thepresence of infection and associated them with demographicvariables, pathological history related to an increased riskof developing severe COVID-19, the doses of rituximabreceived, the type of ventilatory support, thromboembolicevents, and the treatment received. A descriptive analysis ofall the variables was carried out and infected and uninfectedpatients were compared.Results. We screened a total of 68 patients who had received rituximab (median cumulative dose: 4,161mg (2,611–8,187.5)). 54.4% men, mean age 60.8 years (15.7; 25-87)). C +was confirmed for 22 patients. Of these, 45.5% had high bloodpressure, 36.4% Diabetes Mellitus, 31.8% smokers/ex-smoker,22.7% lung disease, 13.6% heart disease and 4.5% obesity.There were no statistically significant differences between C+and C-. Only 2 patients developed immunity. For 10 patients(45.5%) did not have a negative CRP until the end of the follow-up. There was no association with cumulative dose ofrituximab. The mortality rate was 22.7% in the C+.Conclusions. We observe that the persistence of the infection leads to a worse evolution of COVID-19. The use of alternatives should be considered during the pandemic, becauseof patients with decreased B-cell function may have high riskof fatal progression from COVID-19. (AU)

Neurolinfomatosis de los nervios ciático y mediano como presentación inicial del linfoma de linfocitos B / Sciatic and median nerve neurolymphomatosis as initial presentation of B-cell Lymphoma

La neurolinfomatosis es la infiltración de los nervios craneales o de los nervios y raíces del sistema nervioso periférico por un linfoma, generalmente un linfoma no Hodgkin de linfocitos B. Es poco frecuente como presentación inicial de la enfermedad y puede dar lugar a manifestaciones clínicas extremadamente heterogéneas. Informamos del caso de un hombre de 72 años que presentaba entumecimiento de la mano derecha, debilidad progresiva en ambas extremidades inferiores y pérdida de peso. La PET/TC con 18F-FDG mostró masas suprarrenales hipermetabólicas bilaterales, úlcera gástrica, pequeñas adenopatías hipermetabólicas, captación de médula ósea de focalización múltiple y captación intensa tanto en los nervios ciáticos como en el nervio mediano derecho. La biopsia gástrica y de los ganglios confirmó un linfoma difuso de linfocitos B grandes, del tipo de linfocitos B activados, con una resolución posterior de la captación de los nervios periféricos después de iniciar la quimioterapia.(AU)

Neurolymphomatosis (NL) is the infiltration of cranial nerves or nerves and roots from the peripheral nervous system by lymphoma, usually by B-cell non-Hodgkin's lymphoma. It is uncommon as initial presentation of the disease and can lead to extremely heterogeneous clinical manifestations. We report the case of a 72-year old male who presented with numbness of the right hand, progressive weakness in both lower limbs and weight loss. 18F-FDG PET/CT showed bilateral hypermetabolic adrenal masses, gastric ulcer, small hypermetabolic adenopathies, multiple focal bone marrow uptake and intense uptake in both sciatic nerves and right median nerve. A node and gastric biopsy confirmed diffuse large-B-cell lymphoma, activated B cell type, with posterior resolution of peripheral nerves uptake after beginning chemotherapy.(AU)

Efficacy of belimumab in Primary Sjögren's syndrome: A systematic review / Eficacia del belimumab en el síndrome de Sjögren primario: una revisión sistemática

Objective: To evaluate the efficacy and safety of belimumab in patients with Primary Sjögren's syndrome (pSS). Methods: The search included manuscripts assessing the efficacy or safety of belimumab in patients with pSS (American-European Consensus Criteria 2002) published between 2004 and 2017 in MEDLINE, EMBASE or Cochrane databases. Two reviewers independently selected the articles, extracted data and evaluated the quality of the evidence following Scottish Intercollegiate Guidelines Network (SIGN) recommendation grades. Results: Out of 135 citations, only 3 articles were included. All of them publishing results from the same study at different time points including 28 patients. At week 28 improvement was reported for visual analogue scale (VAS) dryness score and glandular manifestations in 37% and 77% of patients, respectively, which persisted at week 52 (W52). Belimumab was well tolerated and safely administered. Conclusion: Published evidence to determine the efficacy of belimumab in pSS is limited. Belimumab seems to be effective to reduce systemic activity, parotid enlargement, lymphadenopathies, articular manifestation and B cell biomarkers.(AU)

Objetivo: Evaluar la eficacia y la seguridad de belimumab en pacientes con síndrome de Sjögren primario (SSp). Métodos: La búsqueda incluyó manuscritos que evaluaban la eficacia o seguridad de belimumab en pacientes con SSp (Criterios Europeo-Americanos del 2002) publicados entre 2004 y 2007 en MEDLINE, EMBASE o Cochrane database. Dos revisores independientes seleccionaron los artículos, extrajeron los datos y evaluaron la calidad de la evidencia según los grados de recomendación de la Scottish Intercollegiate Guidelines Network (SIGN). Resultados: De 135 artículos se incluyeron 3. Todos publicaban resultados del mismo estudio en diferentes momentos, incluyéndose 28 pacientes. En la semana 28 presentaban una mejoría en la puntuación de sequedad en la escala analógica visual (VAS) y en las manifestaciones glandulares un 37 y 77% de los pacientes, respectivamente, que persistieron en la 52. La administración de belimumab fue segura y bien tolerada. Conclusión: Belimumab parece ser efectivo para reducir la actividad sistémica, el aumento parotídeo, las linfadenopatías, las manifestaciones articulares y los biomarcadores de células B, aunque con evidencia limitada.(AU)

Immune cell infiltration features and related marker genes in lung cancer based on single-cell RNA-seq

Purpose Immune cells in the immune microenvironment of lung cancer have a great impact on the development of lung cancer. Our purpose was to analyze the immune cell infiltration features and related marker genes for lung cancer. Methods Single cell RNA sequencing data of 11,485 lung cancer cells were retrieved from the Gene Expression Omnibus. After quality control and data normalization, cell clustering was performed using the Seurat package. Based on the marker genes of each cell type from the CellMarker database, each cell was divided into G1, G2M, and S phases. Then, differential expression and functional enrichment analyses were performed. CIBERSORT was used to reconstruct immune cell types. Results Following cell filtering, highly variable genes were identified for all cells. 14 cell types were clustered. Among them, CD4 + T cell, B cell, plasma cell, natural killer cell and cancer stem cell were the top five cell types. Up-regulated genes were mainly enriched in immune-related biological processes and pathways. Using CIBERSORT, we identified the significantly higher fractions of naïve B cell, memory CD4 + T cell, T follicular helper cell, T regulatory helper cell and M1 macrophage in lung cancer tissues compared to normal tissues. Furthermore, the fractions of resting NK cell, monocyte, M0 macrophage, resting mast cell, eosinophil and neutrophil were significantly lower in tumor tissues than normal tissues. Conclusion Our findings dissected the immune cell infiltration features and related marker genes for lung cancer, which might provide novel insights for the immunotherapy of lung cancer (AU)

Low percentages of regulatory T cells in common variable immunodeficiency (CVID) patients with autoimmune diseases and its association with increased numbers of CD4+CD45RO+ T and CD21low B cells

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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Aumento de las concentraciones de interferón-1alpha, interleucina-10 y BLyS como biomarcadores de actividad clínica en el lupus eritematoso sistémico / Increased interferon-1alpha, interleukin-10 and BLyS concentrations as clinical activity biomarkers in systemic lupus erythematosus

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

B subset cells in patients with chronic granulomatous disease in a Mexican population

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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Correlation of OX40 ligand on B cells with serum total IgE and IL-4 levels by CD4+ T cells in allergic rhinitis

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

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Increased IRF4 expression in isolated B cells from common variable immunodeficiency (CVID) patients

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

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Infliximab como tratamiento de la enteropatía severa en un paciente con inmunodeficiencia común variable e infección por citomegalovirus / Infliximab as treatment of severe enteropathy in a patient with common variable immunodeficiency and cytomegalovirus infection

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Linfoma intravascular de células B grandes como causa inusual de ictus isquémico / Intravascular large B-cell lymphoma as an unusual cause of ischemic stroke

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)