Azizi Gholamreza, Ghanavatinejad Alireza, Abolhassani Hassan, Yazdani Reza, Rezaei Nima, Mirshafiey Abbas, Aghamohammadi Asghar
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.
Expert Rev Clin Immunol. 2016 Sep;12(9):989-1006. doi: 10.1080/1744666X.2016.1177458. Epub 2016 Apr 28.
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.
原发性免疫缺陷病（PID）是一组超过270种的遗传性异质性疾病，会影响免疫系统体液免疫和细胞免疫的不同组成部分。原发性T细胞免疫缺陷在生命早期影响个体。在大多数情况下，T细胞原发性免疫缺陷表现为T细胞和B细胞联合缺陷。T细胞原发性免疫缺陷病患者易发生危及生命的感染。另一方面，诸如淋巴增殖性疾病、癌症和自身免疫等非感染性并发症似乎与原发性T细胞免疫缺陷有关。各类自身免疫性疾病（器官特异性或全身性疾病）都可能属于此类原发性免疫缺陷病；然而，最常见的自身免疫性疾病是免疫性血小板减少性紫癜（ITP）和自身免疫性溶血性贫血（AIHA）。在本综述中，我们讨论了自身免疫的提出机制，并回顾了每种原发性T细胞免疫缺陷中自身免疫性疾病的文献报道。
