Person: Recio Hoyas, María José
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First Name
María José
Last Name
Recio Hoyas
Affiliation
Universidad Complutense de Madrid
Faculty / Institute
Medicina
Department
Inmunología, Oftalmología y ORL
Area
Inmunología
Identifiers
31 results
Search Results
Now showing 1 - 10 of 31
Item Evaluation of Polysaccharide Typhim Vi Antibody Response as a predictor of Humoral Immunodeficiency in Haematological Malignancies(Clinical Immunology, 2020) Ochoa-Grullón, J. et al.; S. Sánchez-Ramón; Benavente Cuesta, Celina; Pérez López, César; Peña Cortijo, Ascensión; Mateos-Aparicio Morales, Gregoria; Williams, J.L. ; Fernández Arquero, Miguel; Recio Hoyas, María JoséAn increasing healthcare challenge in the management of haematological malignancy (HM) is secondary immunodeficiency. From January 2019, the EMA included the evaluation of specific antibody (Ab) responses to better select patients for immunoglobulin replacement therapy (IgRT). We evaluated Ab responses to pneumococcal and Salmonella typhi pure polysaccharide immunization in a cohort of 42 HM patients and 24 healthy-controls. Pre-post specific Ab concentrations were measured by ELISA at 4 weeks. Globally, significantly lower Typhim Vi (TV) seroprevalence (9%) compared to 23-valent pneumococcal polysaccharide vaccine (PPV) (76%) (p <0.001) was observed. TV non responders (88%) were higher than PPV non responders (62%) (p <0.0001) and correlated better to infectious history. By ROC analysis, pre-post 5-fold TV increase was the best cut-off to discriminate HM with recurrent infections and controls (sensitivity 91%, specificity 100%). Despite the small sample cohort, our results suggest that specific anti-S typhi Ab response is a useful complementary assay in the diagnosis and management decision of SID to HM.Item Inmunodeficiencias congénitas del receptor de antígeno de los linfocitos T(Inmunología, 2013) Mazariegos, Marina S.; Muñoz Ruiz, Miguel; Reiné Gutiérrez, Jesús; Garcillán Goyoaga, Beatriz De; Recio Hoyas, María José; Fernández Malavé, Edgar Gonzalo; Regueiro González-Barros, José RamónLas inmunodeficiencias humanas del TCR son enfermedades autosómicas recesivas con baja prevalencia, caracterizadas por un defecto de expresión del TCR asociado a una linfopenia T selectiva (más leve en el caso de CD3γ, TCRα o CD247, o grave en el caso de CD3δ o CD3??). La ausencia congénita de componentes del TCR tiene un impacto diferencial en el desarrollo y función de los linfocitos T, que depende de la cadena del TCR afectada y de la especie, siendo en algunos casos diferente en los pacientes humanos en comparación con los modelos en ratones. El estudio del inmunofenotipo mediante citometría de flujo, junto con los estudios moleculares, proporciona información esencial para el diagnóstico y el tratamiento, que continúa siendo a día de hoy el trasplante de progenitores hematopoyéticos en los casos asociados a inmunodeficiencia grave.Item Virtualización de la asignatura de Inmunología(III jornada Campus Virtual UCM : Innovación en el Campus Virtual metodologías y herramientas, 2007) Cabanillas, Beatriz; Regueiro González-Barros, José Ramón; Martínez Naves, Eduardo; Recio Hoyas, María José; Gómez Del Moral Martín-Consuegra, Manuel María; Setién Baranda , Esteban Fernando; Fernández-Valmayor Crespo, Alfredo; Fernández-Pampillón Cesteros, Ana María; Merino Granizo, JorgeLa Universidad Complutense de Madrid es competitiva en docencia de Inmunología, no existiendo otra Universidad que cubra todas las diplomaturas y licenciaturas en las que hay carga docente de Inmunología [1]. Estas asignaturas, de pocos créditos pero con un temario muy denso, se beneficiarían de un soporte virtual que diera salida a muchas de las necesidades del alumno. Asimismo se ha comprobado que existe un buen material docente de Inmunología en Internet, aunque la mayoría de los contenidos se encuentran en inglés. Por ello los profesores participantes en el proyecto, con su amplia experiencia en innovación y mejora de la calidad docente [2], consideran que la Inmunología es susceptible de virtualizar con éxito.Item Differential Biological Role of CD3 Chains Revealed by Human Immunodeficiencies(The Journal of Inmunology, 2007) Recio Hoyas, María José; Miguel Angel Moreno Pelayo; Sara S. Kiliç; Alberto C. Guardo; Ozden Sanal; Allende Martínez, Luis Miguel; Verónica Pérez Flores; Angeles Mencía; Silvia Modamio Høybjør; Elena Seoane; Regueiro González-Barros, José RamónThe biological role in vivo of the homologous CD3γ and δ invariant chains within the human TCR/CD3 complex is a matter of debate, as murine models do not recapitulate human immunodeficiencies. We have characterized, in a Turkish family, two new patients with complete CD3γ deficiency and SCID symptoms and compared them with three CD3γ-deficient individuals belonging to two families from Turkey and Spain. All tested patients shared similar immunological features such as a partial TCR/CD3 expression defect, mild αβ and γδ T lymphocytopenia, poor in vitro proliferative responses to Ags and mitogens at diagnosis, and very low TCR rearrangement excision circles and CD45RA+ αβ T cells. However, intrafamilial and interfamilial clinical variability was observed in patients carrying the same CD3G mutations. Two reached the second or third decade in healthy conditions, whereas the other three showed lethal SCID features with enteropathy early in life. In contrast, all reported human complete CD3δ (or CD3ε) deficiencies are in infants with life-threatening SCID and very severe αβ and γδ T lymphocytopenia. Thus, the peripheral T lymphocyte pool was comparatively well preserved in human CD3γ deficiencies despite poor thymus output or clinical outcome. We propose a CD3δ ≫ CD3γ hierarchy for the relative impact of their absence on the signaling for T cell production in humans.Item Inherited human ezrin deficiency impairs adaptive immunity(Journal of Allergy and Clinical Immunology, 2023) García-Solís, Blanca et al.; Rebeca Pérez de Diego; Martínez Martínez, Laura María; Recio Hoyas, María José; Fernández Arquero, Miguel; Sánchez Ramón, Silvia MaríaBackground Inborn errors of immunity (IEI) are a group of monogenic diseases that confer susceptibility to infection, autoimmunity, and cancer. Despite the life-threatening consequences of some IEI, their genetic cause remains unknown in many patients. Objective We investigated a patient with an IEI of unknown genetic etiology. Methods Whole-exome sequencing identified a homozygous missense mutation of the gene encoding ezrin (EZR), substituting a threonine for an alanine at position 129. Results Ezrin is one of the subunits of the ezrin, radixin, and moesin (ERM) complex. The ERM complex links the plasma membrane to the cytoskeleton and is crucial for the assembly of an efficient immune response. The A129T mutation abolishes basal phosphorylation and decreases calcium signaling, leading to complete loss of function. Consistent with the pleiotropic function of ezrin in myriad immune cells, multidimensional immunophenotyping by mass and flow cytometry revealed that in addition to hypogammaglobulinemia, the patient had low frequencies of switched memory B cells, CD4+ and CD8+ T cells, MAIT, γδ T cells, and centralnaive CD4+ cells. Conclusions Autosomal-recessive human ezrin deficiency is a newly recognized genetic cause of B-cell deficiency affecting cellular and humoral immunity.Item Human congenital T-cell receptor disorders(LymphoSign Journal, 2015) Marín Marín, Ana Victoria; Garcillán Goyoaga, Beatriz De; Jiménez Reinoso, Anaïs; Muñoz Ruiz, Miguel; Briones Contreras, Alejandro; Fernández Malavé, Edgar Gonzalo; Recio Hoyas, María José; Regueiro González-Barros, José RamónImmunodeficiencies of most T-cell receptor (TCR) components (TCRID) have been reported in almost 40 patients worldwide who have also, at times, shown signs of autoimmunity. We updated their clinical, immunological, and molecular features with an emphasis on practical diagnosis, as the range of the disorder grows in complexity with new partial defects. Cellular and animal models are also reviewed and in some cases reveal their limitations for predicting TCRID immunopathology.Item Natural killer cell hyporesponsiveness and impaired development in a CD247-deficient patient(Journal of Allergy and Clinical Immunology, 2016) Valés Gómez, Mar; Esteso, Gloria; Aydogmus, Cigdem; Blázquez Moreno, Alfonso; Marín Marín, Ana Victoria; Briones Contreras, Alejandro; Garcillán Goyoaga, Beatriz de; García Cuesta, Eva; López Cobo, Sheila; Haskologlu, Sule; Moraru, Manuela; Cipe, Funda E; Dobbs, Kerry; Dogu, Figen; Parolini, Silvia; Notarangelo, Luigi D.; Vilches, Carlos; Recio Hoyas, María José; Regueiro González-Barros, José Ramón; Ikinciogullari, Aydan; Reyburn, Hugh T. RItem DNA damage response and breast cancer development: Possible therapeutic applications of ATR, ATM, PARP, BRCA1 inhibition(DNA Repair, 2021) Mirza-Aghazadeh-Attari, Mohammad; Ghazizadeh Darband, Saber; Mojtaba Kaviani; Safa, Amin; Mihanfar, Ainaz; Sadighparvar, Shirin; Karimian, Ansar; Alemi, Forough; Majidinia, Maryam; Yousefi, Bahman; Recio Hoyas, María JoséBreast cancer is the most common and significant cancers in females regarding the loss of life quality. Similar to other cancers, one of the etiologic factors in breast cancer is DNA damage. A plethora of molecules are responsible for sensing DNA damage and mediating actions which lead to DNA repair, senescence, cell cycle arrest and if damage is unbearable to apoptosis. In each of these, aberrations leading to unrepaired damage was resulted in uncontrolled proliferation and cancer. Another cellular function is autophagy defined as a process eliminating of unnecessary proteins in stress cases involved in pathogenesis of cancer. Knowing their role in cancer, scholars have tried to develop strategies in order to target DDR and autophagy. Further, the interactions of DDR and autophagy plus their regulatory role on each other have been focused simultaneously. The present review study has aimed to illustrate the importance of DDR and autophagy in breast cancer according to the related studies and uncover the relation between DDR and autophagy and its significance in breast cancer therapy.Item Measurement of Typhim Vi IgG as a Diagnostic Tool to Determine Anti-polysaccharide Antibody Production Deficiency in Children(Frontiers in Immunology, 2019) Guevara-Hoyer, Kissy et al.; Pérez de Diego, Rebeca; Gil López, Celia; Recio Hoyas, María José; Fernández Arquero, Miguel; Ramos Amador, José Tomás; Sánchez Ramón, Silvia MaríaBackground: The assessment of specific polysaccharide antibody production plays a pivotal role in the diagnosis of humoral primary immunodeficiencies (PID). The response to 23-valent pneumococcal vaccine (PPV) remains the gold standard for the diagnosis of polysaccharide antibodies. However, in Spain, the interpretation of pure polysaccharide 23-valent immunization is hampered by the high endemicity of pneumococcal disease and the generalization of the 13-valent adjuvant pneumococcal vaccination. Specific Typhim Vi vaccination (TV) immunoglobulin G IgG response to immunization is useful in adult PID, but there is no data regarding children. Objectives: To evaluate the clinical utility of TV IgG production as a diagnostic tool to determine anti-polysaccharide antibody production deficiency in children, when the response to PPV is unclear and isolated determination of serotypes is unfeasible. Methods: We conducted a single-institution prospective observational study on 61 children with recurrent infections. Baseline specific antibodies against PPV and TV were evaluated. In 28 children (46%), the response to the production of antibodies confirmed a clinical suspicion of humoral PID, and they were therefore immunized with 23-valent pneumococcal vaccine and Typhim Vi. Both specific antibody responses were measured by ELISA (The Binding Site Group Ltd, Birmingham, UK) using previously published cut-offs. Results: Seventy percent of the 61 children displayed baseline PPV IgG > 27 mg/L, whereas only 8% showed TV IgG > 28 U/mL (p < 0.0001). Twenty-one of 28 children (75%) achieved a 3-fold increase in post-vaccination TV IgG levels, whereas only 3% achieved a 4-fold increase in PPV IgG post vaccination, mainly due to high baseline PPV IgG titers. When we classified children according to their response to TV as responders or non-responders and compared this with the well-known clinical warning signs of the Jeffrey Modell Foundation. The proportions of children with history of pneumonia and the need for intravenous antibiotics were significantly higher in TV IgG non-responders than in TV IgG responders (p = 0.02 and p = 0.01, respectively). Conclusion: Response to TV can be considered an ancillary diagnostic tool to determine polysaccharide antibodies in children, particularly when isolated determination of pneumococcal serotypes is not feasible. TV provides a useful asset for clinicians in the era of conjugate PPV vaccination, with clinical relevance. Further research is warranted for validation.Item Extreme Phenotypes With Identical Mutations: Two Patients With Same Non-sense NHEJ1 Homozygous Mutation(Frontiers in Immunology, 2019) Dominguez-Pinilla, Nerea; Perrig, Melina Soledad; Rodriguez Vigil-Iturrate, Carmen; Salmón-Rodriguez, Nerea; Martinez Faci, Cristina; Castro-Panete, María J.; Blas-Espada, Javier; López-Nevado, Marta; Ruiz-Garcia, Raquel; Chaparro-García, Rebeca; Recio Hoyas, María José; Allende Martínez, Luis Miguel; González Granado, Luis IgnacioCernunnos/XLF deficiency is a rare primary immunodeficiency classified within the DNA repair defects. Patients present with severe growth retardation, microcephaly, lymphopenia and increased cellular sensitivity to ionizing radiation. Here, we describe two unrelated cases with the same non-sense mutation in the NHEJ1 gene showing significant differences in clinical presentation and immunological profile but a similar DNA repair defect.