Myasthenia gravis complicated by pure red cell aplasia with clonal large granular lymphocytosis in the absence of thymoma: a rare case report and literature review.

IF 5.9 2区 医学 Q1 IMMUNOLOGY Frontiers in Immunology Pub Date : 2025-02-03 eCollection Date: 2025-01-01 DOI:10.3389/fimmu.2025.1367409
Lijun Du, Yiping Liu, Qiaolin Zhou, Fang Xu
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Abstract

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.

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无胸腺瘤的重症肌无力合并单纯红细胞发育不全伴克隆大颗粒淋巴细胞增多1例罕见报告并文献复习。
2013年,一名怀孕早期的年轻女子因呼吸和吞咽困难多次住院。因复发性严重肺部感染终止妊娠。她后来被诊断为重症肌无力(MG)基于阳性乙酰胆碱受体抗体。随后,她的肌肉无力用吡哆斯的明、硫唑嘌呤和强的松得到很好的控制。值得注意的是,在MG诊断后的第七年(2021年),患者出现了严重的贫血(血红蛋白:44 g/L)。骨髓分析显示罕见的纯红细胞发育不全(PRCA)与大颗粒细胞克隆扩增的结合。进一步检查排除胸腺瘤。考虑到药物诱导PRCA的可能性,用他克莫司代替硫唑嘌呤。值得注意的是,贫血在1个月内消失,MG仍得到良好控制。胸腺瘤内异常胸腺增生可改变外周血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、药物和克隆大颗粒T细胞的机制和关联。这个独特的病例强调了MG和PRCA在没有胸腺瘤的情况下前所未有的关联,强调了疾病谱系的复杂性。患者随后于2023年6月成功分娩,为多方面的临床过程增加了另一个维度,值得关注和探索这些疾病之间的潜在联系。
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来源期刊
CiteScore
9.80
自引率
11.00%
发文量
7153
审稿时长
14 weeks
期刊介绍: Frontiers in Immunology is a leading journal in its field, publishing rigorously peer-reviewed research across basic, translational and clinical immunology. This multidisciplinary open-access journal is at the forefront of disseminating and communicating scientific knowledge and impactful discoveries to researchers, academics, clinicians and the public worldwide. Frontiers in Immunology is the official Journal of the International Union of Immunological Societies (IUIS). Encompassing the entire field of Immunology, this journal welcomes papers that investigate basic mechanisms of immune system development and function, with a particular emphasis given to the description of the clinical and immunological phenotype of human immune disorders, and on the definition of their molecular basis.
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