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纯红细胞再生障碍性贫血与重症肌无力:一例无胸腺瘤情况下同时患有这两种自身免疫性疾病的患者。

Pure red cell aplasia and myasthenia gravis: a patient having both autoimmune conditions in the absence of thymoma.

作者信息

Aquilina Annelise, Camilleri David James, Aquilina Josanne

机构信息

Foundation Programme, Mater Dei Hospital, Msida, Malta.

Department of Haematology, Mater Dei Hospital, Msida, Malta.

出版信息

BMJ Case Rep. 2017 Sep 11;2017:bcr-2017-220188. doi: 10.1136/bcr-2017-220188.

Abstract

This is a patient who was presented initially with symptoms of malaise, tiredness and exertional dyspnoea and found to have a severe normocytic normochromic anaemia with low reticulocyte counts. Bone marrow confirmed the diagnosis of pure red cell aplasia (PRCA) and at the time serology for recent parvovirus infection was positive. He was successfully treated with transfusions and intravenous Ig. Six years later, he had a mild relapse of his PRCA and subsequently developed severe dysphagia and dysarthria which were fatigable. Positive antiacetylcholine receptor antibodies confirmed the diagnosis of myasthenia gravis. The two conditions are both known to be associated with thymoma. Imaging and resection of the thymus gland showed only the presence of a thymic cyst. Treatment with pyrdistogmine and intravenous Ig have kept the patient asymptomatic and in remission. The rare association of the two autoimmune conditions associated in the same patient without thymoma is discussed.

摘要

这是一名最初表现为不适、疲倦和劳力性呼吸困难症状的患者,检查发现其患有严重的正细胞正色素性贫血,网织红细胞计数较低。骨髓检查确诊为纯红细胞再生障碍性贫血(PRCA),当时近期细小病毒感染的血清学检查呈阳性。他通过输血和静脉注射免疫球蛋白得到了成功治疗。六年后,他的PRCA出现轻度复发,随后发展为严重的吞咽困难和构音障碍,这些症状会出现疲劳感。抗乙酰胆碱受体抗体阳性确诊为重症肌无力。已知这两种疾病都与胸腺瘤有关。胸腺的影像学检查和切除显示仅存在一个胸腺囊肿。使用吡啶斯的明和静脉注射免疫球蛋白治疗使患者无症状且处于缓解状态。本文讨论了同一患者中这两种自身免疫性疾病在无胸腺瘤情况下罕见的关联。

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Therapy of myasthenia gravis.重症肌无力的治疗
Handb Clin Neurol. 2008;91:253-72. doi: 10.1016/S0072-9752(07)01508-4.
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Acquired pure red cell aplasia: updated review of treatment.获得性纯红细胞再生障碍性贫血:治疗的最新综述
Br J Haematol. 2008 Aug;142(4):505-14. doi: 10.1111/j.1365-2141.2008.07216.x. Epub 2008 May 28.

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