Ramyar Asghar, Aghamohammadi Asghar, Moazzami Kasra, Rezaei Nima, Yeganeh Mehdi, Cheraghi Taher, Pouladi Nima, Heydari Golnaz, Abolhassani Hassan, Amirzargar Ali Akbar, Parvaneh Nima, Moin Mostafa
Department of Pediatrics, Division of Hematology, Children Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Iran J Allergy Asthma Immunol. 2008 Sep;7(3):169-75.
Common Variable Immunodeficiency (CVID) is a heterogeneous group of disorders characterized by hypogammaglobulinemia and an increased susceptibility to recurrent infections as well as autoimmunity and malignancies. Idiopathic Thrombocytopenic Purpura (ITP) and Autoimmune Hemolytic Anemia (AIHA) are two autoimmune disorders which may be seen in association with CVID. Among 85 CVID patients, seven cases had ITP and/or AIHA (8%). Four of these patients had one or more episodes of ITP, one patient had AIHA, and two patients had both ITP and AIHA (Evans syndrome). Almost, all patients experienced chronic and recurrent infections mostly in respiratory and gastrointestinal systems during the course of the disease. Among the seven patients, five presented their underlying disease with recurrent respiratory and/or gastrointestinal tract infections, while in two remaining patients, CVID was presented with ITP. Three patients died until now; two because of hepatic failure and one due to pulmonary hemorrhage. As CVID is prone to autoimmune disorders, it should be considered as a differential diagnosis of adult-onset ITP and possibly in children. Chronic and recurrent ITP, especially in the presence of propensity to respiratory and gastrointestinal infections mandate the evaluation for an underlying immune dysregulation such as CVID.
普通可变型免疫缺陷（CVID）是一组异质性疾病，其特征为低丙种球蛋白血症、反复感染的易感性增加以及自身免疫和恶性肿瘤。特发性血小板减少性紫癜（ITP）和自身免疫性溶血性贫血（AIHA）是两种可能与CVID相关的自身免疫性疾病。在85例CVID患者中，7例患有ITP和/或AIHA（8%）。这些患者中，4例有一次或多次ITP发作，1例患有AIHA，2例同时患有ITP和AIHA（伊文氏综合征）。几乎所有患者在疾病过程中都经历了慢性和反复感染，主要发生在呼吸和胃肠道系统。在这7例患者中，5例以反复呼吸道和/或胃肠道感染为基础疾病，而在其余2例患者中，CVID以ITP为表现。到目前为止，3例患者死亡；2例死于肝衰竭，1例死于肺出血。由于CVID易患自身免疫性疾病，应将其视为成人发病ITP以及可能儿童ITP的鉴别诊断。慢性和反复性ITP，尤其是在存在呼吸道和胃肠道感染倾向的情况下，需要评估是否存在潜在的免疫失调，如CVID。
