title: Recent advances in understanding the pathophysiology of primary T cell immunodeficiencies.
abstract: Primary immunodeficiencies (PIDs) constitute a large group of rare disorders that affect the function of the immune system. These deficiencies are usually associated with inability to prevent infections but can also result in allergy, inflammation, autoimmunity, and cancer. The recent characterization of many different monogenic defects has shed light on a range of non-redundant differentiation, effector, and regulatory functions of immune cells. The present review focuses on PIDs that affect adaptive immunity in general and T lymphocytes in particular. The analysis of PIDs is now opening up new pathways towards targeted therapies.
authors.affiliation: Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, Paris, France; Immunology and Pediatric Hematology Department, Assistance Publique-Hôpitaux de Paris, Paris, France; Institut National de la Santé et de la Recherche Médicale (INSERM) Unité Mixte de Recherche 1163, Paris, France; Collège de France, Paris, France. Electronic address: alain.fischer@inserm.fr.
authors.forename: Alain
authors.initials: A
authors.lastname: Fischer
mesh_terms: D000818:Animals; D015551:Autoimmunity / Q000276:immunology; D006801:Humans; D007113:Immunity, Innate / Q000276:immunology; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D000081207:Primary Immunodeficiency Diseases; D013601:T-Lymphocytes / Q000276:immunology
keywords: T lymphocytes; autoimmunity; inflammation; innate immunity; primary immunodeficiencies