Department of Pathology, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, 325015, China.
Department of Clinical Laboratory , Wenzhou Hospital of Traditional Chinese Medicine Affiliated to Zhejiang Chinese Medical University, 9 Jiaowei Road, Wenzhou, 325000, China.
BMC Immunol. 2021 Aug 3;22(1):50. doi: 10.1186/s12865-021-00441-9.
Good's syndrome (GS) is a rare secondary immunodeficiency disease presenting as thymoma and hypogammaglobulinemia. Due to its rarity, the diagnosis of GS is often missed.
We used the hospital information system to retrospectively screen thymoma and hypogammaglobulinemia patients at the First Affiliated Hospital of Wenzhou Medical University from Apr 2012 to Apr 2020. The clinical, laboratory, treatment, and outcome data for these patients were collected and analyzed.
Among the 181 screened thymoma patients, 5 thymoma patients with hypogammaglobulinemia were identified; 3 patients had confirmed diagnoses of GS, and the other 2 did not have a diagnosis of GS recorded in the hospital information system. A retrospective review of the clinical characteristics, laboratory results, and follow-up data for these 2 undiagnosed patients confirmed the diagnosis of GS. All 5 GS patients presented with pneumonia, 2 patients presented with recurrent skin abscesses, 2 patients presented with recurrent cough and expectoration, 1 patient presented with recurrent oral lichen planus and diarrhea, and 1 patient presented with tuberculosis and granulomatous epididymitis. In the years after the diagnosis of hypogammaglobulinemia with mild symptoms, all 5 patients had received irregular intravenous immunoglobulin (IVIG) treatment. As the course of the disease progressed, the clinical symptoms of all patients worsened, but the symptoms were partly resolved with IVIG in these patients. However, 4 patients died due to comorbidities.
GS should be investigated as a possible diagnosis in thymoma patients who present with hypogammaglobulinemia, especially those with recurrent opportunistic infections, recurrent skin abscesses, chronic diarrhea, or recurrent lichen planus.
Good 综合征（GS）是一种罕见的继发性免疫缺陷病，表现为胸腺瘤和低丙种球蛋白血症。由于其罕见性，GS 的诊断常常被忽视。
我们使用医院信息系统回顾性筛选 2012 年 4 月至 2020 年 4 月温州医科大学附属第一医院的胸腺瘤和低丙种球蛋白血症患者。收集并分析这些患者的临床、实验室、治疗和转归数据。
在筛选出的 181 例胸腺瘤患者中，发现 5 例伴有低丙种球蛋白血症的胸腺瘤患者；其中 3 例确诊为 GS，另外 2 例在医院信息系统中未记录 GS 的诊断。对这 2 例未确诊患者的临床特征、实验室结果和随访数据进行回顾性分析，确认了 GS 的诊断。所有 5 例 GS 患者均表现为肺炎，2 例患者表现为复发性皮肤脓肿，2 例患者表现为复发性咳嗽和咳痰，1 例患者表现为复发性口腔扁平苔藓和腹泻，1 例患者表现为肺结核和肉芽肿性附睾炎。在低丙种球蛋白血症症状较轻的诊断后数年，所有 5 例患者均接受了不规则静脉注射免疫球蛋白（IVIG）治疗。随着疾病的进展，所有患者的临床症状均恶化，但这些患者的症状在接受 IVIG 治疗后部分缓解。然而，有 4 例患者因合并症死亡。
对于伴有低丙种球蛋白血症的胸腺瘤患者，尤其是那些反复发生机会性感染、复发性皮肤脓肿、慢性腹泻或复发性扁平苔藓的患者，应考虑进行 GS 的诊断。
