Fahim A, Abuzakouk M, Hart S P
Division of Cardiovascular and Respiratory Studies, Castle Hill Hospital, Castle Road, Cottingham, United Kingdom.
Rev Port Pneumol. 2011 Nov-Dec;17(6):272-4. doi: 10.1016/j.rppneu.2011.06.002. Epub 2011 Jul 22.
Good's Syndrome is a rare cause of immunodeficiency associated with thymoma. Patients with this syndrome are prone to infections with encapsulated microorganisms. The diagnosis may be delayed for a considerable time period even after the thymectomy.
We describe the case of a 70-year-old woman with a background of thymectomy who presented with pneumonia and gram negative sepsis. Haemophilus influenzae was found in blood cultures. Moreover, there was evidence of impaired B and T cell immunity consistent with Good's Syndrome. She was commenced on immunoglobulin replacement following treatment of sepsis and remains well 18 months after the initial presentation.
This case illustrates the importance of considering Good's Syndrome in the context of pneumonia and immunodeficiency associated with encapsulated organisms such as Haemophilus influenzae. This clinical entity is associated with a significant mortality and should be considered as a cause of immunodeficiency even years after thymectomy.
古德综合征是与胸腺瘤相关的免疫缺陷的罕见病因。该综合征患者易感染包膜微生物。即使在胸腺切除术后,诊断也可能延迟相当长一段时间。
我们描述了一名70岁女性的病例,她有胸腺切除史,出现肺炎和革兰氏阴性菌败血症。血培养发现流感嗜血杆菌。此外,有证据表明B细胞和T细胞免疫受损,符合古德综合征。在治疗败血症后,她开始接受免疫球蛋白替代治疗,首次就诊18个月后情况良好。
该病例说明了在肺炎以及与流感嗜血杆菌等包膜微生物相关的免疫缺陷背景下考虑古德综合征的重要性。这种临床实体与显著的死亡率相关,即使在胸腺切除术后数年,也应被视为免疫缺陷的一个病因。