Good Syndrome is a rare clinical entity first described as the conjunction of thymoma and hypogammaglobulinemia, and more recently depicted as a complex disease integrating a medical history of thymoma with humoral immunodeficiency (more accurately stated: hypogammaglobulinemia) with or without cellular immunodeficiency, recurrent infections, autoimmunity, paraneoplastic syndromes and diverse aberrations in the immunological profile. This condition has an ominous prognosis with a high mortality rate secondary to recalcitrant infectious diseases. Understanding the possible discordances in clinical presentation and the temporal relationship between manifestations and immunological alterations is key to prevent misdiagnosis and complications. To this end, here we provide two illustrative patients with Good Syndrome that share common clinical manifestations and yet show unique and opposed immunological profiles, thereby highlighting the pivotal interest of a comprehensive immunological profiling in these patients. We conducted a thorough review of existing literature on the elusive molecular mechanisms underlying the syndrome and provide a clinical assessment algorithm to facilitate the management of these challenging patients.

良好综合征是一种罕见的临床实体，最初被描述为胸腺瘤和低丙种球蛋白血症的结合，最近被描述为一种复杂的疾病，结合了胸腺瘤病史和体液免疫缺陷（更准确地说：低丙种球蛋白血症），伴有或不伴有细胞免疫缺陷，反复感染，自身免疫、副肿瘤综合征和免疫学特征的各种异常。这种情况具有不祥的预后，具有继发于顽固性传染病的高死亡率。了解临床表现中可能的不一致以及表现与免疫学改变之间的时间关系是防止误诊和并发症的关键。为此，在这里，我们提供了两名具有共同临床表现但表现出独特和相反的免疫学特征的良好综合征患者，从而突出了对这些患者进行全面免疫学分析的关键利益。我们对现有的关于该综合征难以捉摸的分子机制的文献进行了全面审查，并提供了一种临床评估算法，以促进对这些具有挑战性的患者的管理。