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伴纯红细胞再生障碍性贫血、古德综合征及重症肌无力的胸腺瘤对利妥昔单抗治疗有效

Thymoma with Concomitant Pure Red Cell Aplasia, Good's Syndrome and Myasthenia Gravis Responding to Rituximab.

作者信息

Antar Ahmad I, Otrock Zaher K, Kharfan-Dabaja Mohamed A, Mahfouz Rami A, Alameddine Raafat S, El-Majzoub Nadim M W, Salem Ziad M

机构信息

Division of Hematology-Oncology, Department of Internal Medicine, American University of Beirut Medical Center, P.O. Box 11-0236, Beirut, Lebanon.

Department of Pathology and Immunology, Barnes-Jewish Hospital, Washington University, St. Louis, MO USA.

出版信息

Indian J Hematol Blood Transfus. 2016 Jun;32(Suppl 1):219-22. doi: 10.1007/s12288-014-0478-x. Epub 2014 Dec 11.

Abstract

Thymomas are often associated with a variety of autoimmune diseases, mostly myasthenia gravis. The association of thymomas with both pure red cell aplasia (PRCA) and Good's syndrome is exceedingly rare. To the best of our knowledge, the combination of a thymoma with manifestations of myasthenia gravis, PRCA, and Good's syndrome, as in our case herein, has not been described before in the medical literature. We present a 90-year-old man initially diagnosed with an asymptomatic thymoma. Later, he developed generalized muscle weakness and was found to have severe anemia. He was diagnosed with PRCA, myasthenia gravis and Good's syndrome. He responded to rituximab with restoration of bone marrow erythroid maturation and stabilization of red blood cell counts.

摘要

胸腺瘤常与多种自身免疫性疾病相关,其中大多数为重症肌无力。胸腺瘤与纯红细胞再生障碍性贫血(PRCA)和古德综合征同时存在的情况极为罕见。据我们所知,如我们在此病例中所呈现的,胸腺瘤合并重症肌无力、PRCA和古德综合征的表现此前在医学文献中尚未有过描述。我们报告一例90岁男性患者,最初被诊断为无症状胸腺瘤。后来,他出现了全身肌肉无力,并被发现患有严重贫血。他被诊断为PRCA、重症肌无力和古德综合征。他对利妥昔单抗治疗有反应,骨髓红系成熟得以恢复,红细胞计数稳定。

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本文引用的文献

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Thymoma with pure red cell aplasia and Good's syndrome.胸腺瘤伴纯红细胞再生障碍和 Good 综合征。
Ann Thorac Surg. 2011 May;91(5):1620-2. doi: 10.1016/j.athoracsur.2010.10.010.
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Thymoma and autoimmunity.胸腺瘤与自身免疫。
Cell Mol Immunol. 2011 May;8(3):199-202. doi: 10.1038/cmi.2010.74. Epub 2011 Feb 14.
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