胸腺瘤合并純紅細(xì)胞再生障礙性貧血12例臨床報道
發(fā)布時間:2018-07-07 21:29
本文選題:胸腺瘤 + 純紅細(xì)胞再生障礙性貧血 ; 參考:《山東大學(xué)》2017年碩士論文
【摘要】:目的:胸腺瘤(Thymoma)合并純紅細(xì)胞再生障礙性貧血(Pure Red Cell Aplasia,PRCA)在臨床上較為少見,其病理生理機(jī)制亦尚未完全闡明,針對胸腺瘤合并PRCA的治療也沒有統(tǒng)一的標(biāo)準(zhǔn)。本文旨在探討胸腺瘤合并PRCA的臨床表現(xiàn)、診斷、治療及預(yù)后,以提高臨床對該病的認(rèn)識。方法:回顧性分析山東省立醫(yī)院胸外科于2006年6月~2016年6月十年間,所有通過手術(shù)治療的12例胸腺瘤合并PRCA病人的臨床資料及術(shù)后隨訪情況結(jié)果:本組共有12名患者,其中男性患者5名,女性患者7名,沒有發(fā)現(xiàn)明顯的性別偏向。年齡42~71歲,平均年齡58.17歲,中位年齡58.5歲。首次就診均因面色蒼白,感頭暈、乏力等貧血癥狀。12名患者均通過手術(shù)治療,8名患者經(jīng)胸骨正中切口行胸腺切除術(shù),2名患者經(jīng)右胸入路行胸腺切除,2名患者通過電視輔助胸腔鏡手術(shù)(Video-assisted Thoracoscopic Surgery,VATS)切除胸腺。所有 12 名患者均通過手術(shù)完整切除腫瘤及周圍組織,術(shù)后死亡0例。術(shù)后組織病理學(xué)示:AB型3例,B1型3例,B2型4例,B3型2例。對本組病例進(jìn)行Masaoka分期,其中Ⅰ期8例,Ⅱ期3例,Ⅳ期1例。所有12例患者通過電話隨訪,共隨訪11例,失訪1例,隨訪時間從2016年6月至2016年12月。截止隨訪結(jié)束,7名患者達(dá)到治愈標(biāo)準(zhǔn),3名患者死亡,1例截至隨訪時,維持輸血依賴。在達(dá)到治愈的7名患者中,2例術(shù)后未接受任何其他輔助治療,1例單獨用強的松治療,1例接受抗胸腺細(xì)胞球蛋白(Antithymocyte Globulin,ATG)和強的松共同治療,其余3例接受ATG和環(huán)孢素共同治療,并在所有治療停止后維持治愈狀態(tài)。維持輸血依賴的患者在術(shù)后曾接受環(huán)孢素治療,并可脫離輸血維持Hb濃度穩(wěn)定,維持1年后,因藥物毒性不得不降低劑量,患者PRCA復(fù)發(fā)。3例死亡患者,2例死于感染(1例膿胸,1例支氣管擴(kuò)張合并肺炎),1例死于轉(zhuǎn)移性胸腺瘤。結(jié)論:胸腺瘤合并PRCA較為少見;其預(yù)后主要取決于PRCA的治療效果;胸腺瘤并發(fā)的PRCA可能是由免疫因素引起;胸腺切除術(shù)可作為首選治療方案,但僅有小部分患者在胸腺切除后PRCA可以得到治愈,多數(shù)患者在手術(shù)后仍需其他治療手段;類固醇、環(huán)孢素和ATG是較為有效的內(nèi)科治療手段,但可導(dǎo)致較為嚴(yán)重的并發(fā)癥,需對患者進(jìn)行嚴(yán)格監(jiān)測。
[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,
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