Division of Respiratory Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Kobe, Japan.
Division of Pathology, Department of Pathology, Kobe University Graduate School of Medicine, Kobe, Japan.
Kobe J Med Sci. 2020 Jan 20;65(4):E114-E117.
Pulmonary lymphangioleiomyomatosis accounts for the majority of cadaveric lung transplantation cases. Post-transplantation management is continuingly necessary not only to prevent the progression of LAM but also to address complications. A woman with lymphangioleiomyomatosis underwent cadaveric lung transplantation. She developed post-operative native lung hyperinflation and hemoptysis with cavity shadow in the native lung on computed tomography. Isolated Aspergillus from her sputum and positive Aspergillus galactomannan antigen in the blood led to a diagnosis of aspergillosis. Despite the reduction of hemoptysis by antifungal medication, she developed fatal hemoptysis. An autopsy showed an Aspergillus fungal mass in the bronchus in the native lung whilst the lung graft was free from lymphangioleiomyomatosis lesions. Endobronchial aspergilloma was suggested to be a cause of hemoptysis. This fatal clinical course suggested that hemoptysis due to endobronchial aspergilloma in the native lung should have been considered native lung pneumonectomy as a further intervention.
肺淋巴管平滑肌瘤病占尸肺移植病例的大多数。不仅需要进行移植后管理以预防 LAM 的进展,还需要解决并发症。一名淋巴管平滑肌瘤病患者接受了尸肺移植。她在术后出现了原发性肺过度充气和咯血,胸部 CT 显示原发性肺有空腔阴影。她的痰液中分离出了曲霉菌,血液中曲霉菌半乳甘露聚糖抗原呈阳性,诊断为曲霉菌病。尽管抗真菌药物减少了咯血,但她还是出现了致命性咯血。尸检显示原发性肺支气管中有曲霉菌团块,而移植肺未出现淋巴管平滑肌瘤病病变。提示支气管内曲霉菌瘤是咯血的原因。这种致命的临床过程表明,应考虑对原发性肺行肺切除术以进一步干预,因为原发性肺内的支气管内曲霉菌瘤导致了咯血。