【作者】 姚宇锋；

【导师】 柴莹；

【作者基本信息】 浙江大学 ， 外科学， 2004， 硕士

【摘要】 目的 探讨胸腺瘤合并单纯红细胞再生障碍性贫血的发病机理、临床表现、诊断方法和有效的治疗方法。方法 总结从1998年到2002年间胸腺瘤合并单纯红细胞再生障碍性贫血(pure red cell aplasia PRCA)病例4例的临床资料，并检索近年中英文文献进行回顾。结果 本组病人全部接受胸骨正中切口行胸腺瘤扩大切除术，无手术死亡，早期效果很明显。术后随访以无PRCA复发视为有效，结果2年内均无PRCA复发。结论 胸腺瘤合并PRCA是一种自身免疫性疾病，其诊断可以通过胸片，胸部CT和骨髓穿刺活检而确立，胸腺瘤扩大切除是治疗本病的主要方法，术后应辅以激素治疗。由于T淋巴细胞膜表面的CD34抗原和生长因子受体有关，因此基于生长抑制素类似物(octreotide)的药物治疗可能对顽固性的病例有效。更多还原

【Abstract】 Objective: to understand the pathogenesis , clinical features, methodes of diagnosis of thymoma associated with pure red cell aplasia (PRCA) and to evaluate the outcome of the therapy. Methods: The clinical data of 4 cases of thymoma associated with PRCA from 1993 to 2002 and English , Chinese literature of recentyears were reviewed. Result: all of the 4 cases underwent extended thymusectomy and there was no postoperative death.The early results of extended thymusectomy was excellent. The standard of effective therapy: no recurrence of PRCA was observed in 2 years after operation.Conclusion: thymoma associated with PRCA is an autoimmune disease (AID).Bone marrow examination, radiological including CT scan examination is necessary to establish the diagnosis and extended thymusectomy after preparation with repeated blood transfusion remains the mainstay of treatment. Prolonged corticosteroid therapy leads to remission for upto 2 years after operative intervention.Suspected that it isconnected with the CD34 antigen expression on the membrane of T-cell, for CD34 antigen support the function of growth factor receptor, somatostatin analogue-based therapy ( octreotide ) may be an effective therapy in unresponsive thymomas.更多还原