TY - JOUR AU - López-Nevado, Marta AU - González-Granado, Luis I. AU - Ruiz-García, Raquel AU - Pleguezuelo, Daniel AU - Cabrera-Marante, Oscar AU - Salmón, Nerea AU - Blanco-Lobo, Pilar AU - Domínguez-Pinilla, Nerea AU - Rodríguez-Pena, Rebeca AU - Sebastián, Elena AU - Cruz-Rojo, Jaime AU - Olbrich, Peter AU - Ruiz-Contreras, Jesús AU - Paz-Artal, Estela AU - Neth, Olaf AU - Allende, Luis M. PY - 2021 DO - 10.3389/fimmu.2021.671755 UR - http://hdl.handle.net/10668/3632 T2 - Frontiers in Immunology AB - Primary immune regulatory disorders (PIRD) are associated with autoimmunity, autoinflammation and/or dysregulation of lymphocyte homeostasis. Autoimmune lymphoproliferative syndrome (ALPS) is a PIRD due to an apoptotic defect in Fas-FasL pathway and... LA - en PB - Frontiers KW - ALPS KW - ALPS-like KW - Autoimmunity KW - Lymphoproliferation KW - Malignancy KW - Immune dysregulation KW - Síndrome linfoproliferativo autoinmune KW - Autoinmunidad KW - Medical Subject Headings::Diseases::Hemic and Lymphatic Diseases::Lymphatic Diseases::Lymphoproliferative Disorders::Autoimmune Lymphoproliferative Syndrome KW - Medical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Early Diagnosis KW - Medical Subject Headings::Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans KW - Medical Subject Headings::Chemicals and Drugs::Biological Factors::Antigens::Antigens, Surface::Antigens, Differentiation::Antigens, Differentiation, T-Lymphocyte::CTLA-4 Antigen KW - Medical Subject Headings::Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Membrane Proteins::Receptors, Cell Surface::Receptors, Immunologic::Receptors, Natural Killer Cell::Receptors, NK Cell Lectin-Like::NK Cell Lectin-Like... KW - Medical Subject Headings::Phenomena and Processes::Immune System Phenomena::Immunity::Autoimmunity KW - Medical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Diagnostic Techniques and Procedures::Monitoring, Physiologic TI - Primary Immune Regulatory Disorders With an Autoimmune Lymphoproliferative Syndrome-Like Phenotype: Immunologic Evaluation, Early Diagnosis and Management TY - research article ER -