BACKGROUND Thymomas are known to be associated with myasthenia gravis and Good syndrome. Good syndrome is the association of thymoma with combined B cell and T cell immunodeficiency. The combination of all three diseases has not been reported. We discuss the therapeutic dilemma of immunosuppression in such a case. CASE PRESENTATION A 27-year-old Sinhalese man was evaluated for persistent cough which was associated with pleuritic chest pain and was found to have pleural-based lesions in his left hemithorax. Further evaluation confirmed these lesions to be implants from a thymoma. He subsequently developed myasthenia gravis and impending myasthenic crisis precipitated by pneumonia. He was found to have hypogammaglobulinemia with low B cell counts, confirming a diagnosis of Good syndrome. Treatment with intravenously administered broad-spectrum antibiotics, acetylcholinesterase inhibitors, orally administered glucocorticoids, plasma exchange, and intravenous immunoglobulin led to clinical improvement. He subsequently underwent thymectomy and debulking of the tumor and was maintained on regular intravenous immunoglobulins combined with low-dose prednisolone. CONCLUSIONS Regular intravenous immunoglobulins combined with low-dose immunosuppression in addition to thymectomy appear to be safe when myasthenia gravis occurs in association with Good syndrome.

中文翻译：

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胸腺瘤并发重症肌无力和古德综合征——治疗难题：病例报告。


背景技术已知胸腺瘤与重症肌无力和古德综合征有关。 Good 综合征是胸腺瘤与 B 细胞和 T 细胞联合免疫缺陷的关联。这三种疾病同时发生的情况尚未有报道。我们讨论这种情况下免疫抑制的治疗困境。病例介绍 一名 27 岁的僧伽罗男子因持续咳嗽而接受评估，该咳嗽与胸膜炎性胸痛有关，并发现其左半胸有胸膜病变。进一步的评估证实这些病变是胸腺瘤的植入物。随后，他患上了重症肌无力，并因肺炎引发了即将发生的肌无力危机。他被发现患有低丙种球蛋白血症和 B 细胞计数低，证实了 Good 综合征的诊断。静脉注射广谱抗生素、乙酰胆碱酯酶抑制剂、口服糖皮质激素、血浆置换和静脉注射免疫球蛋白治疗导致临床改善。随后，他接受了胸腺切除术和肿瘤减灭术，并继续定期静脉注射免疫球蛋白和低剂量泼尼松龙。结论 当发生与 Good 综合征相关的重症肌无力时，除了胸腺切除术外，常规静脉注射免疫球蛋白联合低剂量免疫抑制剂似乎是安全的。