Lai Yi Wye, Tan Teck-Choon
Tan Tock Seng Hospital, Internal Medicine, Singapore.
Khoo Teck Puat Hospital, Division of Rheumatology, Allergy and Immunology, Department of General Mediine, Singapore.
Asia Pac Allergy. 2020 Oct 20;10(4):e37. doi: 10.5415/apallergy.2020.10.e37. eCollection 2020 Oct.
Good syndrome (GS) is a primary immunodeficiency (PID) that presents in middle aged to older adults with features of thymoma, hypogammaglobulinemia, CD4 T lymphopenia, inverted CD4/CD8+ ratio, and impaired T-cell mitogen proliferative responses. We present a patient, a 62-year-old female, who first presented with disease manifestation of acute hepatitis from hepatitis B virus (HBV) reactivation, which was subsequently complicated by recurrent hospitalizations for recurrent pneumonia and concomitant and cytomegalovirus enteritis. She was later found to have thymoma and hypogammaglobulinemia and was diagnosed with GS. Although the well-known importance of T cell is in directing B-cell responses in the immunopathology of thymoma, low levels of natural killer and CD4+ γδ T cells may also be the cause of both low immune surveillance of tumor development and weak clearance of viral infection. Hence, the temporal sequence of opportunistic infections following HBV reactivation and thymoma discovery may reflect a loss of immune surveillance as the first manifestation of PID.
古德综合征（GS）是一种原发性免疫缺陷病（PID），多见于中老年人，伴有胸腺瘤、低丙种球蛋白血症、CD4 T淋巴细胞减少、CD4/CD8 +比值倒置以及T细胞丝裂原增殖反应受损等特征。我们报告一例62岁女性患者，最初表现为乙型肝炎病毒（HBV）再激活导致的急性肝炎，随后因反复肺炎和巨细胞病毒性肠炎反复发作而多次住院。后来发现她患有胸腺瘤和低丙种球蛋白血症，被诊断为GS。虽然T细胞在胸腺瘤免疫病理学中指导B细胞反应的重要性已广为人知，但自然杀伤细胞和CD4 +γδT细胞水平较低也可能是肿瘤发生的免疫监视低下和病毒感染清除能力减弱的原因。因此，HBV再激活后机会性感染与发现胸腺瘤的时间顺序可能反映了作为PID首发表现的免疫监视丧失。
