Hashiro Wakana, Miyashita Akihiro, Kawaji-Kanayama Yuka, Okamoto Haruya, Fujino Takahiro, Tsukamoto Taku, Mizutani Shinsuke, Shimura Yuji, Kuroda Junya
Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, Kyoto, JPN.
Cureus. 2024 May 4;16(5):e59654. doi: 10.7759/cureus.59654. eCollection 2024 May.
Good's syndrome is a pathologic condition characterized by thymoma and immunoglobulin disorder. Here, we report a rare case of a patient with Good's syndrome with simultaneous pure red cell aplasia (PRCA) and subclinical myasthenia gravis with detectable serum anti-acetylcholine receptor antibody (AChR Ab). While thymectomy did not result in the improvement of any paraneoplastic syndromes, cyclosporine A (CsA) treatment successfully improved PRCA; however, hypoglobulinemia was not recovered, and anti-AchR Ab did not disappear by CsA treatment in our case. A review of the literature on simultaneous Good's syndrome with PRCA also suggested the efficacy of CsA on PRCA but not hypoglobulinemia, suggesting the distinct underlying mechanisms between these two paraneoplastic symptoms with thymoma. Future research is needed to understand the mechanism underlying this rare pathologic condition and to generate appropriate treatment.
古德综合征是一种以胸腺瘤和免疫球蛋白紊乱为特征的病理状况。在此，我们报告一例罕见的古德综合征患者，该患者同时患有纯红细胞再生障碍性贫血（PRCA）和亚临床重症肌无力，且血清中可检测到抗乙酰胆碱受体抗体（AChR Ab）。虽然胸腺切除术并未使任何副肿瘤综合征得到改善，但环孢素A（CsA）治疗成功改善了PRCA；然而，在我们的病例中，低球蛋白血症未恢复，且CsA治疗后抗AchR Ab并未消失。对同时患有PRCA的古德综合征的文献综述也表明，CsA对PRCA有效，但对低球蛋白血症无效，这表明这两种胸腺相关性副肿瘤症状的潜在机制不同。需要进一步研究以了解这种罕见病理状况的潜在机制并制定合适的治疗方法。
