Sun Shuxiang, Zhang Yuanfeng, Ma Junjie, Chen Liming, Wang Yan
Department of Hematology, Yantai Yuhuangding Hospital Affiliated to Qingdao University, 20 Yuhuangding East Road, Shandong Province, 264000, Yantai, China.
J Cardiothorac Surg. 2026 Feb 1;21(1):109. doi: 10.1186/s13019-026-03859-7.
Thymoma-related aplastic anemia is a rare entity. This article retrospectively analyzes the clinical features and treatment course of a patient who developed aplastic anemia (AA) post-thymectomy, complemented by a systematic review of relevant literature. A 47-year-old female was diagnosed with thymoma, myasthenia gravis (MG), and severe AA (SAA). SAA onset occurred two weeks after total thymectomy, and the patient ultimately succumbed to concurrent COVID-19 infection following allogeneic hematopoietic stem cell transplantation (allo-HSCT). We also reviewed the clinical characteristics, treatment strategies, and prognosis of 47 thymoma-related aplastic anemia patients reported in the literature. AA may present prior to thymoma diagnosis, concurrently with thymoma, or post-thymectomy. Some patients progress to pure red cell aplasia (PRCA) and/or megakaryocytic aplasia, often following prior chemotherapy or radiotherapy. Similar to Good syndrome and PRCA, thymectomy fails to alleviate AA, and spontaneous improvement is rare. Treatment options for thymoma-related aplastic anemia include cyclosporine A (CsA) monotherapy, CsA combined with glucocorticoids, thrombopoietin receptor agonists (TPO-RAs), and allo-HSCT. However, regimens of cyclophosphamide plus methylprednisolone and glucocorticoid monotherapy show limited efficacy. The overall one-year mortality rate is alarmingly high at 29.8%. For young thymoma-related aplastic anemia patients with SAA and suitable donors, allo-HSCT remains the preferred treatment.
胸腺瘤相关再生障碍性贫血是一种罕见的病症。本文回顾性分析了一名患者胸腺切除术后发生再生障碍性贫血（AA）的临床特征及治疗过程，并对相关文献进行了系统综述。一名47岁女性被诊断为胸腺瘤、重症肌无力（MG）和重型再生障碍性贫血（SAA）。SAA在全胸腺切除术后两周发病，该患者在异基因造血干细胞移植（allo-HSCT）后最终死于并发的新型冠状病毒肺炎（COVID-19）感染。我们还回顾了文献报道的47例胸腺瘤相关再生障碍性贫血患者的临床特征、治疗策略及预后。AA可能在胸腺瘤诊断之前、与胸腺瘤同时出现或在胸腺切除术后出现。一些患者在先前化疗或放疗后常进展为纯红细胞再生障碍性贫血（PRCA）和/或巨核细胞再生障碍。与Good综合征和PRCA相似，胸腺切除术无法缓解AA，自发改善也很罕见。胸腺瘤相关再生障碍性贫血的治疗选择包括环孢素A（CsA）单药治疗、CsA联合糖皮质激素、血小板生成素受体激动剂（TPO-RAs）以及allo-HSCT。然而，环磷酰胺加甲泼尼龙方案和糖皮质激素单药治疗的疗效有限。总体一年死亡率高达29.8%，令人担忧。对于患有SAA且有合适供体的年轻胸腺瘤相关再生障碍性贫血患者，allo-HSCT仍然是首选治疗方法。
