胸腺瘤合并纯红细胞再生障碍性贫血12例临床报道

发布时间：2018-07-07 21:29

  本文选题：胸腺瘤 + 纯红细胞再生障碍性贫血　； 参考：《山东大学》2017年硕士论文

【摘要】：目的:胸腺瘤(Thymoma)合并纯红细胞再生障碍性贫血(Pure Red Cell Aplasia,PRCA)在临床上较为少见,其病理生理机制亦尚未完全阐明,针对胸腺瘤合并PRCA的治疗也没有统一的标准。本文旨在探讨胸腺瘤合并PRCA的临床表现、诊断、治疗及预后,以提高临床对该病的认识。方法:回顾性分析山东省立医院胸外科于2006年6月～2016年6月十年间,所有通过手术治疗的12例胸腺瘤合并PRCA病人的临床资料及术后随访情况结果:本组共有12名患者,其中男性患者5名,女性患者7名,没有发现明显的性别偏向。年龄42～71岁,平均年龄58.17岁,中位年龄58.5岁。首次就诊均因面色苍白,感头晕、乏力等贫血症状。12名患者均通过手术治疗,8名患者经胸骨正中切口行胸腺切除术,2名患者经右胸入路行胸腺切除,2名患者通过电视辅助胸腔镜手术(Video-assisted Thoracoscopic Surgery,VATS)切除胸腺。所有 12 名患者均通过手术完整切除肿瘤及周围组织,术后死亡0例。术后组织病理学示:AB型3例,B1型3例,B2型4例,B3型2例。对本组病例进行Masaoka分期,其中Ⅰ期8例,Ⅱ期3例,Ⅳ期1例。所有12例患者通过电话随访,共随访11例,失访1例,随访时间从2016年6月至2016年12月。截止随访结束,7名患者达到治愈标准,3名患者死亡,1例截至随访时,维持输血依赖。在达到治愈的7名患者中,2例术后未接受任何其他辅助治疗,1例单独用强的松治疗,1例接受抗胸腺细胞球蛋白(Antithymocyte Globulin,ATG)和强的松共同治疗,其余3例接受ATG和环孢素共同治疗,并在所有治疗停止后维持治愈状态。维持输血依赖的患者在术后曾接受环孢素治疗,并可脱离输血维持Hb浓度稳定,维持1年后,因药物毒性不得不降低剂量,患者PRCA复发。3例死亡患者,2例死于感染(1例脓胸,1例支气管扩张合并肺炎),1例死于转移性胸腺瘤。结论:胸腺瘤合并PRCA较为少见;其预后主要取决于PRCA的治疗效果;胸腺瘤并发的PRCA可能是由免疫因素引起;胸腺切除术可作为首选治疗方案,但仅有小部分患者在胸腺切除后PRCA可以得到治愈,多数患者在手术后仍需其他治疗手段;类固醇、环孢素和ATG是较为有效的内科治疗手段,但可导致较为严重的并发症,需对患者进行严格监测。
[Abstract]:Objective: Pure Red Cell aplastic anemia (PRCA) is rare in clinic, its pathophysiological mechanism has not been fully elucidated, and there is no uniform standard for the treatment of thymoma with PRCA. The purpose of this article is to investigate the clinical manifestations, diagnosis, treatment and prognosis of thymoma complicated with PRCA in order to improve the clinical understanding of the disease. Methods: the clinical data and follow-up results of 12 patients with thymoma complicated with PRCA from June 2006 to June 2016 in Shandong Provincial Hospital were retrospectively analyzed. There were 5 male patients and 7 female patients, and no obvious gender bias was found. The average age was 58.17 years with a median age of 58.5 years. The first visit was due to pale face, feeling dizzy, All 12 patients with anemic symptoms were treated by operation. Two patients underwent thymectomy via the right thoracic approach and two patients underwent video-assisted Thoracoscopic surgery via video-assisted thoracoscopic surgery (Video-assisted thoracoscopic surgery vats). All 12 patients underwent complete resection of the tumor and surrounding tissues, and 0 patients died after operation. Postoperative histopathology showed 3 cases of type B _ 1 and 3 cases of B _ 1 type B _ 2 and 2 cases of B _ 3 type B _ 3. Masaoka stage was performed in 8 cases of stage 鈪，

本文编号：2106332