Ishii Tomoya, Bandoh Shuji, Kanaji Nobuhiro, Tadokoro Akira, Watanabe Naoki, Imataki Osamu, Dobashi Hiroaki, Kushida Yoshio, Haba Reiji, Yokomise Hiroyasu
Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Japan.
Intern Med. 2016;55(2):105-11. doi: 10.2169/internalmedicine.55.4539. Epub 2016 Jan 15.
Objective Air-leak syndrome (ALS) is a life-threatening pulmonary complication following allogeneic bone marrow transplantation (allo-BMT) which is thought to be associated with graft-versus-host disease (GVHD). Recently, it has been reported that pleuroparenchymal fibroelastosis (PPFE) also occurs after allo-BMT and often causes ALS. We sought to extract common features of ALS caused by PPFE after allo-BMT. Methods The clinical data of patients who developed ALS caused by PPFE after undergoing allo-BMT (ALS-PPFE) between April 1996 and December 2007 at our institution were collected and reviewed retrospectively. The clinical findings, radiological and pathological features and treatment outcomes of ALS-PPFE were assessed. Results Five patients who developed ALS had histologically proven PPFE (four men, one woman: median age, 37 years). The age of onset of ALS-PPFE was 13 to 109 months (median, 68.8 months) after BMT. Alkylating agents were used as conditioning chemotherapy for BMT in all patients. Only one patient developed chronic GVHD (limited type). The common radiological findings were subpleural thickening and traction bronchiectasis predominantly in the bilateral upper lung fields. The histological pulmonary specimens showed no findings of bronchiolitis obliterans or GVHD. Immunosuppressive therapy was not effective in any of the cases, and all patients died of respiratory failure with or without lung transplantation. Conclusion ALS-PPFE is an extremely late-onset noninfectious pulmonary complication of allo-BMT. This complication is progressive, resistant to immunosuppressive treatment and has a poor prognosis. No association was found between PPFE and GVHD.
目的 空气泄漏综合征（ALS）是异基因骨髓移植（allo - BMT）后一种危及生命的肺部并发症，被认为与移植物抗宿主病（GVHD）有关。最近，有报道称胸膜实质纤维弹性组织增生症（PPFE）在allo - BMT后也会发生，且常导致ALS。我们试图提取allo - BMT后由PPFE引起的ALS的共同特征。方法 回顾性收集并分析了1996年4月至2007年12月在我院接受allo - BMT后发生由PPFE引起的ALS（ALS - PPFE）患者的临床资料。评估了ALS - PPFE的临床表现、影像学和病理学特征以及治疗结果。结果 5例发生ALS的患者经组织学证实为PPFE（4例男性，1例女性；中位年龄37岁）。ALS - PPFE的发病时间为BMT后13至109个月（中位时间68.8个月）。所有患者均使用烷化剂作为BMT的预处理化疗药物。仅1例患者发生慢性GVHD（局限型）。常见的影像学表现为双侧上肺野为主的胸膜下增厚和牵拉性支气管扩张。肺组织学标本未显示闭塞性细支气管炎或GVHD的表现。免疫抑制治疗对所有病例均无效，所有患者均死于呼吸衰竭，无论是否接受肺移植。结论 ALS - PPFE是allo - BMT一种极其迟发的非感染性肺部并发症。这种并发症呈进行性发展，对免疫抑制治疗耐药，预后不良。未发现PPFE与GVHD之间存在关联。
