Du Lijun, Liu Yiping, Zhou Qiaolin, Xu Fang
Department of Haematology, Mianyang Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Mianyang, China.
Front Immunol. 2025 Feb 3;16:1367409. doi: 10.3389/fimmu.2025.1367409. eCollection 2025.
In 2013, a young woman during her early pregnancy was repeatedly hospitalized due to respiratory and swallowing difficulties. The pregnancy was terminated due to recurrent severe lung infections. She was later diagnosed with myasthenia gravis (MG) based on positive acetylcholine receptor antibodies. Her muscle weakness was subsequently well-controlled with pyridostigmine bromide, azathioprine, and prednisone. Notably, in the seventh year after her MG diagnosis (2021), the patient developed severe anemia (hemoglobin: 44 g/L). Bone marrow analysis revealed a rare combination of pure red cell aplasia (PRCA) with clonal expansion of large granular cells. Further examinations excluded thymoma. Considering the possibility of drug-induced PRCA, azathioprine was replaced with tacrolimus. Remarkably, the anemia resolved within 1 month, and her MG remained well-controlled. It is well-established that abnormal thymic hyperplasia within thymomas can alter the distribution and function of peripheral T lymphocytes, leading to the development of autoimmune diseases such as MG and PRCA. In this unique case without thymoma, we discussed the mechanisms and associations of PRCA with MG, medication, and clonal large granular T cells. This unique case highlights the unprecedented association of MG and PRCA without thymoma, underscoring the complexity of the disease spectrum. The patient's subsequent successful delivery in June 2023 adds another dimension to the multifaceted clinical course, warranting attention and exploration into potential connections between these conditions.
2013年,一名年轻女性在孕早期因呼吸和吞咽困难多次住院。由于反复发生严重肺部感染,此次妊娠终止。后来她因乙酰胆碱受体抗体呈阳性被诊断为重症肌无力(MG)。随后,她的肌无力通过溴吡斯的明、硫唑嘌呤和泼尼松得到了很好的控制。值得注意的是,在她被诊断为MG后的第七年(2021年),该患者出现了严重贫血(血红蛋白:44 g/L)。骨髓分析显示纯红细胞再生障碍(PRCA)与大颗粒细胞克隆性扩增的罕见组合。进一步检查排除了胸腺瘤。考虑到药物性PRCA的可能性,将硫唑嘌呤换成了他克莫司。值得注意的是,贫血在1个月内得到缓解,她的MG仍得到很好的控制。众所周知,胸腺瘤内异常的胸腺增生可改变外周T淋巴细胞的分布和功能,导致MG和PRCA等自身免疫性疾病的发生。在这个没有胸腺瘤的独特病例中,我们讨论了PRCA与MG、药物治疗以及克隆性大颗粒T细胞之间的机制和关联。这个独特的病例突出了MG和PRCA在无胸腺瘤情况下前所未有的关联,强调了疾病谱的复杂性。该患者随后于2023年6月成功分娩,为这一多方面的临床病程增添了新层面,值得关注并探索这些情况之间的潜在联系。