Good综合症是一种罕见的成年起病免疫缺陷，其特点是胸腺瘤、低γ球蛋白血症和易感染的反复发作。临床表现极其多样化，范围从反复细菌和机会感染到同时存在的自身免疫性疾病和有时恶性病理。由于临床表型的多样性和缺乏充分的诊断标准，其识别往往具有挑战性，甚至有时会延迟多年。它是免疫缺陷综合征中最不常见且研究较少的形式之一，其病理生理机制仍不清楚。最初认为它是胸腺瘤相关的原发性抗体缺陷的变异形式，具有成熟B细胞数量减少或缺失，但后来已经明确，T细胞介导的免疫功能的重要缺陷是机会性感染的潜在原因。根据目前的证据，Good综合症被评为一种独特的获得性混合免疫缺陷状态，并被归类为原发性免疫缺陷疾病的表型副本。表观遗传和获得性基因因素可能在其演变中起到最终作用。© 2023作者。由斯堪的纳维亚病理学、医学微生物学和免疫学学会代表John Wiley & Sons有限公司出版的APMIS出版。

Good's syndrome, an infrequent adult-onset immunodeficiency is characterized by the triad of thymoma, hypogammaglobulinemia, and increased susceptibility to recurrent infections. The clinical presentation is highly variable, with a spectrum ranging from recurrent bacterial and opportunistic infections to concomitant autoimmune diseases and, sometimes malignant pathologies. Due to heterogeneous clinical phenotypes and the lack of adequate diagnostic criteria, its recognition is often challenging, even delaying it by years. It is one of the most unusual, less studied form of the immune deficiency syndromes with a still unknown pathophysiology. It was initially considered a thymoma-associated variant of primary antibody deficiencies with a reduced or absent number of mature B cells, but it later emerged that significant defects of T cell-mediated immune functions are the underlying cause of opportunistic infections. On the basis of current evidence, Good's syndrome is evaluated as a distinct acquired form of combined immunodeficiency states and classified as a phenocopy of primary immunodeficiency diseases. Epigenetic and acquired genetic factors can play an ultimate role in its evolution.© 2023 The Authors. APMIS published by John Wiley & Sons Ltd on behalf of Scandinavian Societies for Pathology, Medical Microbiology and Immunology.