Lung transplantation in treatment of secondary pleuroparenchymal fibroelastosis: report of one case and literature review
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摘要:
目的 探讨胸膜肺弹力纤维增生症(PPFE)的治疗策略。 方法 1例男性22岁患者,因淋巴瘤行化学药物治疗及干细胞移植后并发PPFE,行胸腔镜下左肺舌叶楔形切除术、双侧胸膜固定术后,再接受同种异体左肺移植术。检索相关文献,对PPFE的病因及发病机制、临床表现、影像学特征、病理特征和治疗进行分析。 结果 本例PPFE患者肺移植术前需24 h依赖无创呼吸机,接受肺移植治疗后气促消失、呼吸衰竭治愈,生活质量明显提高。国内数据库中未发现符合条件文献,国外数据库中检索出英文文献26篇,经过筛选最终纳入9篇,均为个案报道。PPFE根据病因可分为原发性及继发性,主要临床表现为干咳、劳累性呼吸困难、胸痛、反复气胸及体质量下降,胸部CT可见双上肺胸膜不规则增厚,病理表现为脏层胸膜显著增厚,胸膜及其下肺间质弹力纤维增生、排列紊乱。PPFE病情进展较快,肾上腺皮质激素及其他免疫抑制剂治疗效果差,预后差,需行肺移植治疗。 结论 PPFE内科治疗效果欠佳,情况允许下应尽早予肺移植治疗。 Abstract:Objective To explore the treatment strategies of pleuroparenchymal fibroelastosis (PPFE). Methods A 22-year-old male patient was complicated with PPFE after receiving chemotherapy in combination with stem cell transplantation for lymphoma. He underwent thoracoscopic left lung tongue wedge resection, bilateral pleurodesis followed by allogeneic left lung transplantation. Literature review was performed to analyze the etiology, pathogenesis, imaging features, pathological features and treatment of PPFE. Results The PPFE patient required the non-invasive ventilator for 24 h before lung transplantation. After lung transplantation, the shortness of breath and respiratory failure were cured and the quality of life was significantly improved. No eligible studies was found in the domestic database, and 26 literatures published in English were found in the international databases. Among them, 9 literatures (case reports) were finally included after screening. PPFE could be divided into the primary and secondary categories according to the etiology. The clinical manifestations of PPFE mainly included dry cough, dyspnea on exertion, chest pain, repeated pneumothorax and body weight loss. Chest CT scan demonstrated irregular thickening of the pleura in bilateral upper lungs. Pathological manifestations consisted of evident thickening of the visceral pleura, fibroelastosis and arrangement disorder in the pleura and the underlying pulmonary interstitium. PPFE could progress rapidly. Adrenocortical hormone and other immunosuppressive agents yielded low clinical efficacy and poor clinical prognosis. Lung transplantation was a necessary treatment for PPFE. Conclusions PPFE cannot be effectively treated by conservative therapy. It is recommended to deliver lung transplantation as early as possible. -
表 1 肺移植治疗PPFE相关文献检索结果
Table 1. Relevant literature retrieval results of lung transplantation for the treatment of PPFE
研究作者 年份 国家 性别 年龄(岁) 既往史 临床表现 确诊至移植时间 肺移植方式 存活时间 Chen F, et al[7] 2014 日本 男 28 淋巴细胞白血病
环磷酰胺治疗气胸 7年 左肺 >4个月 Portillo K, et al[8] 2015 西班牙 男 25 Castleman病 呼吸困难
干咳
体质量下降14个月 双肺 >2年 Ishii T, et al[9] 2016 日本 男 27 骨髓增生异常综合征
异基因干细胞移植术后发热 13个月 - 9个月 Hata A, et al[10] 2016 日本 男 19 淋巴细胞白血病
环磷酰胺治疗干咳
胸痛4年 活体肺叶 - Yanagiya M, et al[11] 2016 日本 女 27 - 呼吸困难
扁平胸3个月 活体肺叶 >6个月 Gomes PS, et al[12] 2017 巴西 男 29 禽类接触史
慢性过敏性肺炎呼吸困难
干咳
体质量下降1年 - - Cha YJ, et al[13] 2017 韩国 男 52 肺结核
肺真菌病呼吸困难 2年 双肺 - Huang H, et al[14] 2017 中国 男 34 - 咳嗽
呼吸困难3年 双肺 >6个月 Shimada A, et al[15] 2018 日本 女 21 淋巴瘤
异基因干细胞移植呼吸困难 2年 活体
肺叶>1年 -为研究中未提及 -
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