Department of Clinical Immunology, IML and IdSSC, Hospital Clínico San Carlos, Madrid, Spain.
Department of Medical Oncology, Hospital Clínico San Carlos, Madrid, Spain.
Immunol Lett. 2021 Mar;231:11-17. doi: 10.1016/j.imlet.2020.12.010. Epub 2021 Jan 5.
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.
Good 综合征是一种罕见的临床病症，最初被描述为胸腺瘤和低丙种球蛋白血症的联合病症，最近又被描述为一种复杂的疾病，将胸腺瘤的病史与体液免疫缺陷（更准确地说是低丙种球蛋白血症）与或不伴有细胞免疫缺陷、复发性感染、自身免疫、副肿瘤综合征和免疫谱的多种异常结合在一起。由于难治性传染病，这种情况预后不良，死亡率高。了解临床表现中的可能差异以及表现和免疫改变之间的时间关系是防止误诊和并发症的关键。为此，我们在这里提供两个具有 Good 综合征的说明性患者，他们具有共同的临床表现，但表现出独特且相反的免疫学特征，从而突出了对这些患者进行全面免疫学分析的重要性。我们对该综合征潜在的难以捉摸的分子机制进行了全面的文献回顾，并提供了一种临床评估算法，以帮助管理这些具有挑战性的患者。
