留言板

尊敬的读者、作者、审稿人, 关于本刊的投稿、审稿、编辑和出版的任何问题, 您可以本页添加留言。我们将尽快给您答复。谢谢您的支持!

姓名
邮箱
手机号码
标题
留言内容
验证码

肺移植治疗继发性胸膜肺弹力纤维增生症1例并文献复习

练巧燕 陈奥 徐鑫 韦兵 罗群 顾莹莹 陈荣昌 巨春蓉 何建行

练巧燕, 陈奥, 徐鑫, 等. 肺移植治疗继发性胸膜肺弹力纤维增生症1例并文献复习[J]. 器官移植, 2019, 10(2): 192-197. doi: 10.3969/j.issn.1674-7445.2019.02.013
引用本文: 练巧燕, 陈奥, 徐鑫, 等. 肺移植治疗继发性胸膜肺弹力纤维增生症1例并文献复习[J]. 器官移植, 2019, 10(2): 192-197. doi: 10.3969/j.issn.1674-7445.2019.02.013
Lian Qiaoyan, Chen Ao, Xu Xin, et al. Lung transplantation in treatment of secondary pleuroparenchymal fibroelastosis: report of one case and literature review[J]. ORGAN TRANSPLANTATION, 2019, 10(2): 192-197. doi: 10.3969/j.issn.1674-7445.2019.02.013
Citation: Lian Qiaoyan, Chen Ao, Xu Xin, et al. Lung transplantation in treatment of secondary pleuroparenchymal fibroelastosis: report of one case and literature review[J]. ORGAN TRANSPLANTATION, 2019, 10(2): 192-197. doi: 10.3969/j.issn.1674-7445.2019.02.013

肺移植治疗继发性胸膜肺弹力纤维增生症1例并文献复习

doi: 10.3969/j.issn.1674-7445.2019.02.013
基金项目: 

国家重点实验室建设项目计划 SKLRD-QN-201710

广东省自然科学基金 2018A030313107

详细信息
    作者简介:

    练巧燕,女,硕士研究生,研究方向为肺移植术后并发症,Email:dryan1110@126.com

    通讯作者:

    巨春蓉,女,主任医师,副教授,硕士研究生导师,研究方向为终末期肺疾病肺移植术前评估与术后并发症诊治,Email:juchunrong@126.com

    何建行,男,主任医师,教授,博士研究生导师,研究方向为胸部微创外科与胸部器官移植,Email:hejx@vip.163.com

  • 中图分类号: R617, R322.3+5

Lung transplantation in treatment of secondary pleuroparenchymal fibroelastosis: report of one case and literature review

More Information
  • 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。

     

  • 图  1  本例PPFE患者术前和术后胸部CT表现

    A、B图为术前肺窗及纵隔窗,可见两肺透亮度减低,肺纹理增粗、紊乱,胸膜增厚,病变主要位于双上肺;C、D图为术前肺窗及纵隔窗,可见双上肺多发斑片状模糊影、磨玻璃密度增高影及纤维条索影,伴牵拉性支气管扩张;E、F图为左肺移植术后6个月,G、H图为肺移植术后1年半,可见左移植肺膨胀良好,右侧胸廓塌陷,右肺容积逐渐缩小

    Figure  1.  Preoperative and postoperative chest CT manifestations of the patient with PPFE

    图  2  本例PPFE患者肺组织病理学变化

    A图为脏层胸膜增厚,肺泡间隔稍增宽,间质散在淋巴细胞浸润(苏木素-伊红,×40);B图为胸膜及胸膜下肺组织弹力纤维稍增生紊乱、局部断裂(弹力纤维染色,×100)

    Figure  2.  Pathological changes of lung tissue of the patient with PPFE

    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 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 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 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 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 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 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 52 肺结核
    肺真菌病
    呼吸困难 2年 双肺 -
    Huang H, et al[14] 2017 中国 34 - 咳嗽
    呼吸困难
    3年 双肺 >6个月
    Shimada A, et al[15] 2018 日本 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
    下载: 导出CSV
  • [1] PORTILLO K, GUASCH ARRIAGA I, RUIZ-MANZANO J.Pleuroparenchymal fibroelastosis:is it also an idiopathic entity?[J]. Arch Bronconeumol, 2015, 51(10):509-514.DOI: 10.1016/j.arbres.2015.05.002.
    [2] KINOSHITA Y, WATANABE K, ISHⅡ H, et al.Proliferation of elastic fibres in idiopathic pulmonary fibrosis:a whole-slide image analysis and comparison with pleuroparenchymal fibroelastosis[J]. Histopathology, 2017, 71(6):934-942.DOI: 10.1111/his.13312.
    [3] MEIGNIN V, THIVOLET-BEJUI F, KAMBOUCHNER M, et al.Lung histopathology of non-infectious pulmonary complications after allogeneic haematopoietic stem cell transplantation[J]. Histopathology, 2018, 73(5):832-842.DOI: 10.1111/his.13697.
    [4] TAKEUCHI Y, MIYAGAWA-HAYASHINO A, CHEN F, et al.Pleuroparenchymal fibroelastosis and non-specific interstitial pneumonia:frequent pulmonary sequelae of haematopoietic stem cell transplantation[J]. Histopathology, 2015, 66(4):536-544.DOI: 10.1111/his.12553.
    [5] SCHRIJVER IT, LUIJK B, MEIJER RCA, et al.Successful treatment of bronchial stenosis after lung transplantation[J]. Interact Cardiovasc Thorac Surg, 2017, 24(6):980-981.DOI: 10.1093/icvts/ivx042.
    [6] LOWTHER CM, MORRISON AO, CANDELARIO NM, et al.Novel cutaneous manifestations of pleuroparenchymal fibroelastosis[J]. Am J Dermatopathol, 2016, 38(10):e140-e143.DOI: 10.1097/DAD.0000000000000664.
    [7] CHEN F, MATSUBARA K, MIYAGAWA-HAYASHINO A, et al.Lung transplantation for pleuroparenchymal fibroelastosis after chemotherapy[J]. Ann Thorac Surg, 2014, 98(5):e115-e117.DOI:10.1016/j.athoracsur.2014. 07.045.
    [8] PORTILLO K, GUASCH I, BECKER C, et al.Pleuroparenchymal fibroelastosis:a new entity within the spectrum of rare idiopathic interstitial pneumonias[J]. Case Rep Pulmonol, 2015:810515.DOI: 10.1155/2015/810515.
    [9] ISHII T, BANDOH S, KANAJI N, et al.Air-leak syndrome by pleuroparenchymal fibroelastosis after bone marrow transplantation[J]. Intern Med, 2016, 55(2):105-111.DOI: 10.2169/internalmedicine.55.4539.
    [10] HATA A, NAKAJIMA T, YOSHIDA S, et al.Living donor lung transplantation for pleuroparenchymal fibroelastosis[J]. Ann Thorac Surg, 2016, 101(5):1970-1972.DOI: 10.1016/j.athoracsur.2015.07.056.
    [11] YANAGIYA M, SATO M, KAWASHIMA S, et al.Flat chest of pleuroparenchymal fibroelastosis reversed by lung transplantation[J]. Ann Thorac Surg, 2016, 102(4):e347-e349.DOI: 10.1016/j.athoracsur.2016.02.092.
    [12] GOMES PS, SHIANG C, SZARF G, et al.Pleuroparenchymal fibroelastosis:report of two cases in Brazil[J]. J Bras Pneumol, 2017, 43(1):72-75.DOI: 10.1590/S1806-37562016000000045.
    [13] CHA YJ, HAN J, CHUNG MP, et al.Pleuroparenchymal fibroelastosis in heterogeneous clinical conditions:clinicopathologic analysis of 7 cases[J]. Clin Respir J, 2018, 12(4):1495-1502.DOI: 10.1111/crj.12696.
    [14] HUANG H, FENG R, LI S, et al.A CARE-compliant case report:lung transplantation for a Chinese young man with idiopathic pleuroparenchymal fibroelastosis[J]. Medicine (Baltimore), 2017, 96(19):e6900.DOI: 10.1097/MD.0000000000006900.
    [15] SHIMADA A, TERADA J, TSUSHIMA K, et al.Veno-venous extracorporeal membrane oxygenation bridged living-donor lung transplantation for rapid progressive respiratory failure with pleuroparenchymal fibroelastosis after allogeneic hematopoietic stem cell transplantation[J]. Respir Investig, 2018, 56(3):258-262.DOI: 10.1016/j.resinv.2017.12.009.
    [16] TRAVIS WD, COSTABEL U, HANSELL DM, et al.An official American Thoracic Society/European RespiratorySociety statement:update of the international multidisciplinary classification of the idiopathic interstitial pneumonias[J]. Am J Respir Crit Care Med, 2013, 188(6):733-748.DOI: 10.1164/rccm.201308-1483ST.
    [17] WATANABE K.Pleuroparenchymal fibroelastosis:its clinical characteristics[J]. Curr Respir Med Rev, 2013(9):299-237. http://d.old.wanfangdata.com.cn/NSTLQK/NSTL_QKJJ0232176175/
    [18] KINOSHITA Y, WATANABE K, ISHⅡ H, et al.Pleuroparenchymal fibroelastosis as a histological background of autoimmune diseases[J]. Virchows Arch, 2018.DOI:10.1007/s00428-018-2473-3[Epubahead of print].
    [19] WATANABE S, WASEDA Y, TAKATO H, et al.Pleuroparenchymal fibroelastosis:distinct pulmonary physiological features in nine patients[J]. Respir Investig, 2015, 53(4):149-155.DOI: 10.1016/j.resinv.2015.02.003.
    [20] BONIFAZI M, MONTERO MA, RENZONI EA.Idiopathic pleuroparenchymal fibroelastosis[J]. Curr Pulmonol Rep, 2017, 6(1):9-15.DOI: 10.1007/s13665-017-0160-5.
    [21] KHIROYA R, MACALUSO C, MONTERO MA, et al.Pleuroparenchymal fibroelastosis:a review of histopathologic features and the relationship between histologic parameters and survival[J]. Am J Surg Pathol, 2017, 41(12):1683-1689.DOI: 10.1097/PAS.0000000000000928.
    [22] NAMKOONG H, ISHII M, MORI T, et al.Clinical and radiological characteristics of patients with late-onset severe restrictive lung defect after hematopoietic stem cell transplantation[J]. BMC Pulm Med, 2017, 17(1):123.DOI: 10.1186/s12890-017-0466-7.
  • 加载中
图(2) / 表(1)
计量
  • 文章访问数:  315
  • HTML全文浏览量:  207
  • PDF下载量:  14
  • 被引次数: 0
出版历程
  • 收稿日期:  2019-01-08
  • 网络出版日期:  2021-01-19
  • 刊出日期:  2019-03-15

目录

    /

    返回文章
    返回