Mizobuchi S, Yamashiro T, Nonami Y, Yamamoto A, Kume M, Nakaya H, Sawada T, Taguchi H, Moriki T, Ogoshi S
Department of Surgery II, Kochi Medical School, Japan.
Jpn J Clin Oncol. 1998 Nov;28(11):696-701. doi: 10.1093/jjco/28.11.696.
A case of pure red cell aplasia (PRCA), myasthenia gravis (MG) and thymoma is reported. A 70-year-old woman presented with severe anemia. She had been diagnosed as having MG 8 years earlier and her symptoms were adequately controlled with ambenonium chloride. When she visited our hospital, her hematocrit was 13.7% with a hemoglobin concentration of 4.7 g/dl and her reticulocyte counts were persistently abnormal at 0.1%. Although both direct and indirect Coombs' tests were positive, there was no evidence of hemolysis. Routine screening tests for other etiologies of anemia were negative. Serological tests for anti-DNA and anti-acetylcholine receptor antibodies gave positive results. A bone marrow examination revealed severe erythroid hypoplasia. PRCA was diagnosed and the patient was treated with periodic transfusions. A lateral view chest roentgenogram and a computed tomography scan of the thorax showed the presence of an anterior mediastinal mass which was suspected to be thymoma. The patient underwent thymothymectomy and the tumor was diagnosed as a thymoma. Although the patient received no treatment for MG and PRCA after surgery, her hematological test results rapidly improved and she was discharged from the hospital on the 29th postoperative day. At that time, her hematocrit was 33.2%, her hemoglobin concentration was 10.0 g/dl, her peripheral reticulocyte level was 1.8% and her left partial ptosis had improved. She is doing well, 9 months after surgery. For a patient to remain in remission without treatment for PRCA and MG after thymothymectomy is extremely rare.
报告了一例纯红细胞再生障碍性贫血（PRCA）、重症肌无力（MG）合并胸腺瘤的病例。一名70岁女性因严重贫血就诊。她8年前被诊断为MG，症状通过氯化阿伯农得到有效控制。她来我院就诊时，血细胞比容为13.7%，血红蛋白浓度为4.7g/dl，网织红细胞计数持续异常，为0.1%。虽然直接和间接抗人球蛋白试验均为阳性，但无溶血证据。贫血其他病因的常规筛查试验均为阴性。抗DNA和抗乙酰胆碱受体抗体的血清学检查呈阳性。骨髓检查显示严重的红系造血低下。诊断为PRCA，患者接受定期输血治疗。胸部X线侧位片和胸部计算机断层扫描显示前纵隔有一肿块，怀疑为胸腺瘤。患者接受了胸腺切除术，肿瘤被诊断为胸腺瘤。虽然患者术后未接受MG和PRCA的治疗，但其血液学检查结果迅速改善，并于术后第29天出院。当时，她的血细胞比容为33.2%，血红蛋白浓度为10.0g/dl，外周血网织红细胞水平为1.8%，左侧上睑下垂有所改善。术后9个月，她情况良好。胸腺切除术后未经治疗的PRCA和MG患者仍处于缓解状态极为罕见。
