Good's Syndrome Presenting with Bronchiectasis and Recurrent Pulmonary Infection: A Case Report

Good’s症候群（免疫缺乏合併胸腺瘤）是一種少見的疾病，僅發生在約百分之十的成人型低免疫球蛋白病人。臨床上病人都發現有免疫及血液系統的缺陷，其表徵有貧血、下痢、反覆性肺部及機會性感染，如念珠菌食道炎等。本報告為一位48歲男性病患，從1997年就有支氣管擴張症和反覆肺部感染，甚至因此呼吸衰竭而需機械性通氣。在2000年10月，病人被發現有前縱隔腔腫塊，經細針抽取的細胞學抹片証實為胸腺瘤。免疫檢查發現全免疫球蛋白低下，CD19細胞缺乏，CD4、CD8細胞減少和低比例的CD4/CD8細胞。當病人有胸腺瘤，且合併支氣管擴張症和反覆性肺部及機會性感染病史時，Good’s症候群應被懷疑。

關鍵字

胸線瘤；低免疫球蛋白症；免疫不全；反覆感染

並列摘要

Good’s syndrome (immunodeficiency associated with thymoma) is a rare condition which occurs in only about 10% of patients with adult onset hypogammaglobulinemia. Patients reported in the literature develop defects in both the immune and hematopoietic systems with the clinical features of anemia, diarrhea, and recurrent pulmonary and opportunistic infections such as esophageal candidiasis, and others. We herein report a 48-year-old male with a history of bronchiectasis and recurrent pulmonary infection, beginning in 1997, which required mechanical ventilation at one time due to respiratory failure from infectious complications. In Nov. 2000, he was found to have an enlarged anterior mediastinal mass with a needle aspiration-proven thymoma. Immunological testing showed pan-hypogammaglobulinemia, a depletion of CD 19 cells, and decreased CD4 & CD8 cells with a low CD4/ CD8 ratio, indicating the presence of combined immune deficiency. Good’s syndrome (GS) should be suspected when a patient with thymoma has a history of bronchiectasis combined with recurrent pulmonary or opportunistic infection.

並列關鍵字

thymoma ； hypogammaglobulinemia ； immunodificiency ； recurrent infection

國際替代計量

Good's Syndrome Presenting with Bronchiectasis and Recurrent Pulmonary Infection: A Case Report

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