Department of Medicine, Division of Infectious Diseases, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA.
Clin Immunol. 2010 Jun;135(3):347-63. doi: 10.1016/j.clim.2010.01.006. Epub 2010 Feb 10.
Good syndrome (GS) is a rare association of thymoma and immunodeficiency first described more than 50 years ago. However, this syndrome still remains a mystery to clinicians. We systematically reviewed all the clinical, laboratory and immunologic findings from 152 patients with Good syndrome. The syndrome has a worldwide distribution and approximately half of the cases (47%) have been described in Europe. The diagnosis of thymoma preceded the diagnosis of hypogammaglobulinemia, infection, or diarrhea in 42% of patients whereas in 38% of patients the diagnoses were made almost simultaneously within 2 months of each other. We found significant mortality in patients with this syndrome (44.5%). Astute clinical acumen and increased awareness about the clinical and immunological profile of this syndrome may increase early recognition of this syndrome and prevent mortality. Further studies are needed to elucidate this clinical entity.
Good 综合征（GS）是一种罕见的胸腺瘤和免疫缺陷的关联，早在 50 多年前就有描述。然而，这种综合征对临床医生来说仍然是一个谜。我们系统地回顾了 152 例 Good 综合征患者的所有临床、实验室和免疫学发现。该综合征分布于世界各地，约有一半（47%）的病例发生在欧洲。42%的患者中，胸腺瘤的诊断先于低丙种球蛋白血症、感染或腹泻，而 38%的患者的诊断几乎同时发生，彼此之间相差 2 个月。我们发现患有这种综合征的患者死亡率很高（44.5%）。敏锐的临床洞察力和对这种综合征的临床和免疫学特征的认识提高，可能会增加对这种综合征的早期识别，并降低死亡率。需要进一步的研究来阐明这种临床实体。
