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胸腺瘤合并纯红细胞再生障碍性贫血及重症肌无力的外科治疗

[Surgery for the thymoma combined with pure red cell aplasia and myasthenia gravis].

作者信息

Ayabe T, Matsuzaki Y, Edagawa M, Shimizu T, Hara M, Tomita M, Akiyama Y, Onitsuka T

机构信息

Department of Second Surgery, Miyazaki Medical College, University of Miyazaki, Miyazaki, Japan.

出版信息

Kyobu Geka. 2005 Nov;58(12):1023-9; discussion 1029-31.

Abstract

UNLABELLED

Pure red cell aplasia (PRCA) and myasthenia gravis (MG) are respectively combined with thymoma, however, these 3 complications are extremely rare coexisted as a clinical triad. A 73-year-old female with mediastinal tumor found in 2000 was pointed out anemia in June 2002. As PRCA was diagnosed by the bone marrow examination, blood transfusion had been performed. By a chest computed tomography (CT), a thymoma in size of 7 x 5 cm in diameter was recognized in the anterior mediastinum. The serum level of anti-acetylcholine receptor antibody was elevated to be 35 nmol/l. MG was simultaneously diagnosed with a decreased power of neck muscle. The extended thymectomy was performed in August 2002, and pathological diagnosis disclosed a 'type AB' by World Health Organization (WHO) classification. After the operation, the decreased power of neck muscle had been improved, however, PRCA had not been remitted in the early-postoperative term. Blood transfusion had been required (2-4 units/1-2 weeks) for the postoperative 7 months' term. A cyclosporin (250 mg/day) as an adjuvant therapy was administered in April 2003. One month later, the patient's serum level of Hb had been over 10 g/dl without blood transfusion. The patient has been followed up with reducing the dose of cyclosporin.

CONCLUSIONS

Surgery for a thymoma combined with PRCA and MG was effective for MG but not for PRCA in an early-postoperative term, however, a multimodality therapy with immunosuppressant as a postoperative adjuvant should bring a favorable outcome to patient's clinical data, and the postoperative long-observation must be critical in this case.

摘要

未标注

纯红细胞再生障碍性贫血(PRCA)和重症肌无力(MG)分别与胸腺瘤相关,然而,这三种并发症以临床三联征形式同时存在极为罕见。一名73岁女性于2000年发现纵隔肿瘤,2002年6月出现贫血。经骨髓检查诊断为PRCA,遂进行输血治疗。胸部计算机断层扫描(CT)显示前纵隔有一个直径7×5cm的胸腺瘤。抗乙酰胆碱受体抗体血清水平升高至35nmol/l。同时诊断为MG,伴有颈部肌肉力量减弱。2002年8月行扩大胸腺切除术,病理诊断按世界卫生组织(WHO)分类为“A B型”。术后,颈部肌肉力量减弱有所改善,但术后早期PRCA未缓解。术后7个月期间需要输血(2 - 4单位/1 - 2周)。2003年4月给予环孢素(250mg/天)作为辅助治疗。1个月后,患者血清血红蛋白水平在未输血情况下超过10g/dl。随后对患者进行随访并逐渐减少环孢素剂量。

结论

胸腺瘤合并PRCA和MG的手术对MG有效,但术后早期对PRCA无效,然而,术后使用免疫抑制剂作为辅助的多模式治疗应为患者的临床资料带来良好结果,并且在这种情况下术后长期观察至关重要。

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