title: Autoimmunity in primary T-cell immunodeficiencies.
abstract: Primary immunodeficiency diseases (PID) are a genetically heterogeneous group of more than 270 disorders that affect distinct components of both humoral and cellular arms of the immune system. Primary T cell immunodeficiencies affect subjects at the early age of life. In most cases, T-cell PIDs become apparent as combined T- and B-cell deficiencies. Patients with T-cell PID are prone to life-threatening infections. On the other hand, non-infectious complications such as lymphoproliferative diseases, cancers and autoimmunity seem to be associated with the primary T-cell immunodeficiencies. Autoimmune disorders of all kinds (organ specific or systemic ones) could be subjected to this class of PIDs; however, the most frequent autoimmune disorders are immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA). In this review, we discuss the proposed mechanisms of autoimmunity and review the literature reported on autoimmune disorder in each type of primary T-cell immunodeficiencies.
authors.affiliation: a Department of Laboratory Medicine , Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences , Karaj , Iran.|b Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Gholamreza
authors.initials: G
authors.lastname: Azizi
authors.affiliation: c Department of Immunology, School of Public Health , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Alireza
authors.initials: A
authors.lastname: Ghanavatinejad
authors.affiliation: b Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.|d Division of Clinical Immunology, Department of Laboratory Medicine , Karolinska Institute at Karolinska University Hospital Huddinge , Stockholm , Sweden.
authors.forename: Hassan
authors.initials: H
authors.lastname: Abolhassani
authors.affiliation: e Department of Immunology, School of Medicine , Isfahan University of Medical Sciences , Isfahan , Iran.
authors.forename: Reza
authors.initials: R
authors.lastname: Yazdani
authors.affiliation: b Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
authors.affiliation: c Department of Immunology, School of Public Health , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Abbas
authors.initials: A
authors.lastname: Mirshafiey
authors.affiliation: b Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
mesh_terms: D000744:Anemia, Hemolytic, Autoimmune / Q000453:epidemiology / Q000276:immunology*; D000818:Animals; D015551:Autoimmunity; D001402:B-Lymphocytes / Q000276:immunology*; D006801:Humans; D007108:Immune Tolerance; D007153:Immunologic Deficiency Syndromes / Q000453:epidemiology / Q000276:immunology*; D000081207:Primary Immunodeficiency Diseases; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000453:epidemiology / Q000276:immunology*; D013601:T-Lymphocytes / Q000276:immunology*
keywords: Primary immunodeficiency; T cell; autoimmunity; regulatory T cell; tolerance