低丙种球蛋白血症
胸腺瘤
医学
免疫缺陷
自身免疫
免疫学
免疫失调
恶性肿瘤
重症肌无力
原发性免疫缺陷
常见可变免疫缺陷
免疫系统
病理
抗体
作者
Chen Wang,Yiyun Shi,Xin Rong Lim,Jolán E. Walter
标识
DOI:10.1016/j.ejim.2024.04.006
摘要
Good syndrome (GS) is an acquired adult-onset combined immunodeficiency typically characterized by thymoma and hypogammaglobulinemia. Other clinical manifestations include recurrent infections, autoimmunity, and secondary malignancy [1]. However, the spectrum of acquired immunodeficiency observed in patients with thymoma is more diverse and heterogeneous than previously recognized, extending beyond the classic presentation of hypogammaglobulinemia. The impaired expression of autoimmune regulator (AIRE) in thymoma plays a crucial role in the development of immune dysregulation in these patients, given its critical function in central T cell education and autoreactive clone elimination.
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