Good's Syndrome (Thymoma and Immunodeficiency): Report of 2 Unique Cases and a Literature Review

古德氏症候群（胸腺瘤合併免疫低下）－病例報告

許淳翔(Chun-Hsiang Hsu) ； 林文仁(Wen-Jen Lin) ； 賴瑞生(Ruay-Sheng Lai)

《胸腔醫學》 32卷5期 (2017/10) Pp. 213-219

摘要

Good’s syndrome is defined as a thymoma with T-cell and B-cell immunodeficiency. Patients with Good’s syndrome are susceptible to encapsulated bacterial, viral, fungal, and other opportunistic infections, and also frequently have autoimmune manifestations, such as myasthenia gravis, pure red cell aplasia, and other hematological abnormalities. Here, we report 2 cases of Good’s syndrome with unique presentations: 1 patient presented with Kaposi sarcoma, the other with myelodysplastic syndrome (MDS). Both of our patients suffered from recurrent infections in spite of thymomectomy. One patient received monthly intravenous immunoglobulin replacement therapy, which decreased the number of infections. These cases can enhance our knowledge of a rare but potentially recurrent lethal infectious disease and our understanding of the unique presentations of Good’s syndrome patients.

關鍵字

Good’s syndrome ； thymoma ； immunodeficiency ； intravenous immunoglobulin

並列摘要

古德氏症候群定義為胸腺瘤病患合併T細胞和B細胞免疫缺陷，患者易受夾膜性細菌，病毒，真菌和伺機性病原體的感染。部分患者也有自體免疫的表現，例如重症肌無力，純紅血球再生不良和其他血液學異常。我們報導二位古德氏症候群伴隨特殊表現的病患。對於胸腺瘤合併復發性感染的患者，應進行免疫學檢查，包括淋巴細胞亞群分析和定量免疫球蛋白。早期診斷及治療古德氏症候群對於預後有幫助。適合開刀的胸腺瘤患者應接受胸腺切除術，以防止局部浸潤和遠處轉移。大多數患者在胸腺切除術後，無法改善低丙種球蛋白血症。為了降低各種感染的風險，建議使用預防性靜脈內免疫球蛋白。希望藉由此病例報告能讓臨床醫師增加對此少見疾病的了解。

並列關鍵字

古德氏症候群 ； 胸腺瘤 ； 免疫缺陷 ； 靜脈注射免疫球蛋白