Fijolek Justyna, Wiatr Elzbieta, Demkow Urszula, Orlowsk Tadeusz Maria
Institute of Tuberculosis and Lung Diseases, Warsaw, Poland.
Clin Invest Med. 2009 Aug 1;32(4):E301-6. doi: 10.25011/cim.v32i4.6622.
Immunodeficiency with a thymoma (Good's syndrome) is a rare condition occurring in patients with adult-onset hypogammaglobulinaemia.
We describe the case of a 38-yr-old woman with an upper mediastinal mass and inflammatory infiltrations in the lungs. After thymectomy, the patient's condition did not improve. The HRCT scan showed bronchiectasies with parenchymal opacities. As pulmonary infection persisted despite wide spectrum antibiotic therapy, additional tests were performed to diagnose an immunodeficiency. Serum immunoglobulin levels were very low. T cell response to mitogens was normal, but to Staphylococcus aureus Cowan I was impaired. Immunophenotyping of peripheral blood and bone marrow aspirate showed a very low number of B-cell at all the stages of development (CD10+CD19+, CD5+CD20). In peripheral blood 2.5% of CD19+ lymphocytes were found. On the basis of clinical history and immunological analysis, Good's syndrome was recognized. Treatment with intravenous gammaglobulin and antibiotics improved the patient's performance.
Measurement of serum immunoglobulin concentration is recommended for all patients suspected of thymoma.
伴胸腺瘤的免疫缺陷（古德综合征）是一种发生于成人起病的低丙种球蛋白血症患者的罕见病症。
我们描述了一名38岁女性的病例，该患者有上纵隔肿块及肺部炎性浸润。胸腺切除术后，患者病情未改善。高分辨率CT扫描显示支气管扩张伴实质混浊。尽管进行了广谱抗生素治疗，肺部感染仍持续存在，于是进行了额外检查以诊断免疫缺陷。血清免疫球蛋白水平非常低。T细胞对丝裂原的反应正常，但对金黄色葡萄球菌考恩I株的反应受损。外周血和骨髓穿刺液的免疫表型分析显示，在各个发育阶段的B细胞数量都非常少（CD10+CD19+、CD5+CD20）。在外周血中发现2.5%的CD19+淋巴细胞。根据临床病史和免疫学分析，确诊为古德综合征。静脉注射丙种球蛋白和抗生素治疗改善了患者的状况。
对于所有疑似胸腺瘤的患者，建议检测血清免疫球蛋白浓度。
