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重症肌无力合并胸腺瘤及自身免疫性溶血性贫血。病例报告。

Myasthenia gravis with thymoma and autoimmune haemolytic anaemia. A case report.

作者信息

Tuncer Elmaci N, Ratip S, Ince-Günal D, Tanridağ T

机构信息

Department of Neurology, Marmar University School of Medicine, Istanbul, Turkey.

出版信息

Neurol Sci. 2003 Apr;24(1):34-6. doi: 10.1007/s100720300019.

Abstract

Myasthenia gravis is an autoimmune disorder that affects the neuromuscular junction and leads to weakness of the skeletal muscles. Associated autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis and pernicious anaemia are present in approximately 5% of the myasthenic patients. This report presents a 64-year-old man with autoimmune haemolytic anemia associated with myasthenia gravis and thymoma. The patient developed a severe Coomb's positive autoimmune haemolytic anaemia, which was resistant to treatment with large doses of prednisone. Haemolytic anaemia entered remission one month following thymectomy, and the patient has maintained a normal haemoglobin and a negative Coomb's test without the need for steroid or immunosuppressive therapy. In conclusion, thymectomy may induce a striking improvement of therapyresistant autoimmune haemolytic anemia in patients with MG and thymoma, but in terms of remission, a long follow-up is needed as autoimmune diseases can show spontaneous fluctuations.

摘要

重症肌无力是一种自身免疫性疾病,会影响神经肌肉接头并导致骨骼肌无力。约5%的重症肌无力患者伴有自身免疫性疾病,如系统性红斑狼疮、类风湿性关节炎和恶性贫血。本报告介绍了一名64岁男性,患有与重症肌无力和胸腺瘤相关的自身免疫性溶血性贫血。该患者出现严重的库姆斯试验阳性自身免疫性溶血性贫血,对大剂量泼尼松治疗耐药。胸腺切除术后一个月,溶血性贫血进入缓解期,患者血红蛋白维持正常,库姆斯试验阴性,无需使用类固醇或免疫抑制治疗。总之,胸腺切除术可能会使重症肌无力和胸腺瘤患者的治疗抵抗性自身免疫性溶血性贫血显著改善,但就缓解情况而言,由于自身免疫性疾病可能会出现自发波动,因此需要长期随访。

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