Ye Yucai, Wang Juan, Bao Bahu, Chen Guorong, Hu Aoyan, Sun Jingzi, Liu Weiying
Department of Respiratory Medicine, The First Clinical Medical College of Lanzhou University, Lanzhou, 730000, China.
Department of Respiratory and Critical Care Medicine, the First Hospital of Lanzhou University, No.1 Donggang West Road, Chengguan District, Lanzhou City, 730000, Gansu Province, China.
Immunol Res. 2024 Dec;72(6):1288-1298. doi: 10.1007/s12026-024-09528-y. Epub 2024 Aug 24.
Good syndrome (GS), a rare acquired immunodeficiency disorder characterized by thymoma and hypogammaglobulinemia, predisposes individuals to recurrent infections. This study reports a case of a 37-year-old male GS with multiple pulmonary infections and reviews relevant literature. The patient, with a history of thymoma resection, experienced multiple hospitalizations due to lung infections and neutropenia. The alveolar lavage fluid was detected by macro-genomic sequencing (NGS) to detect multiple pathogens, and targeted anti-infective and immunity-enhancing treatments led to improved symptoms and normal neutrophil counts. A literature review of 98 case reports from 2000 to 2023 was conducted, summarizing the associated diseases and pathogens in GS patients. Regular immunoglobulin monitoring in thymoma patients is essential for early GS diagnosis. When empirical antimicrobial therapy fails, mNGS for pathogen detection and targeted therapy are crucial, and regular IVIG injections can reduce infection rates in GS patients.
古德综合征（GS）是一种罕见的获得性免疫缺陷疾病，其特征为胸腺瘤和低丙种球蛋白血症，易使个体反复发生感染。本研究报告了一例37岁男性古德综合征患者发生多次肺部感染的病例，并对相关文献进行了综述。该患者有胸腺瘤切除病史，因肺部感染和中性粒细胞减少多次住院。通过宏基因组测序（NGS）检测肺泡灌洗液以检测多种病原体，针对性的抗感染和增强免疫力治疗使症状改善且中性粒细胞计数恢复正常。对2000年至2023年的98例病例报告进行了文献综述，总结了古德综合征患者的相关疾病和病原体。对胸腺瘤患者定期监测免疫球蛋白对于早期诊断古德综合征至关重要。当经验性抗菌治疗失败时，采用宏基因组测序检测病原体并进行靶向治疗至关重要，定期静脉注射免疫球蛋白可降低古德综合征患者的感染率。
