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异基因骨髓移植后无慢性移植物抗宿主病的重症肌无力。

Myasthenia gravis without chronic GVHD after allogeneic bone marrow transplantation.

作者信息

Baron F, Sadzot B, Wang F, Beguin Y

机构信息

Department of Medicine, Division of Hematology, University of Liège, Belgium.

出版信息

Bone Marrow Transplant. 1998 Jul;22(2):197-200. doi: 10.1038/sj.bmt.1701297.

Abstract

A 20-year-old man with aplastic anemia developed myasthenia gravis (MG) 7 months after bone marrow transplantation (BMT) from an HLA one locus-mismatched sister. Proximal muscle weakness (predominant in the lower limbs) and dysphagia occurred without any other sign of graft-versus-host disease (GVHD), 1 month after cessation of immunosuppression with cyclosporine. The diagnosis of MG was based on clinical symptoms and on neurophysiologic investigations showing a significant increase of the Jitter in single-fiber electromyography and a significant decremental response during repetitive stimulation at slow rates, but antibodies against the acetylcholine receptor (AchRab) were negative. All clinical and neurophysiological signs normalized within 1 month of treatment with low-dose prednisolone and pyridostigmine, and the patient is perfectly well 1 year after cessation of all therapy. All cases of BMT-associated MG previously published are reviewed in comparison with ours. The originality of this new observation is that this case is the only one not associated with chronic GVHD and negative for AchRab. Alternatively, MG may have been the sole manifestation of chronic GVHD in this patient.

摘要

一名20岁的再生障碍性贫血男性患者,在接受来自一位HLA一个位点不匹配的姐姐的骨髓移植(BMT)7个月后发生了重症肌无力(MG)。停用环孢素免疫抑制1个月后,出现近端肌无力(以下肢为主)和吞咽困难,无任何移植物抗宿主病(GVHD)的其他迹象。MG的诊断基于临床症状以及神经生理学检查,单纤维肌电图显示抖动显著增加,慢速重复刺激时出现显著递减反应,但抗乙酰胆碱受体(AchRab)抗体为阴性。在使用低剂量泼尼松龙和吡啶斯的明治疗1个月内,所有临床和神经生理学体征均恢复正常,在停止所有治疗1年后患者状况良好。将先前发表的所有与BMT相关的MG病例与我们的病例进行了比较。这一新观察结果的独特之处在于,该病例是唯一一例与慢性GVHD无关且AchRab阴性的病例。另外,MG可能是该患者慢性GVHD的唯一表现。

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