Sugimoto Ryujiro, Nakao Atsunori, Yamane Masaomi, Toyooka Shinichi, Okazaki Megumi, Aoe Motoi, Seyama Kuniaki, Date Hiroshi, Oto Takahiro, Sano Yoshifumi
Department of Thoracic Surgery, Okayama University Hospital, Okayama, Japan.
J Heart Lung Transplant. 2008 Aug;27(8):921-4. doi: 10.1016/j.healun.2008.05.012. Epub 2008 Jun 30.
In this study we report the case of a 28-year-old female patient with recurrent lymphangioleiomyomatosis (LAM) in the allografts after bilateral living-donor lobar lung transplantation. Although her post-operative course under immunosuppression with tacrolimus and prednisolone had been uneventful without rejection episodes, she had developed shortness of breath and a progressive chylous effusion with diffuse cystic changes in both lungs 5 years after transplantation. In spite of a diagnosis of having a recurrence of LAM based on radiologic findings and deteriorating pulmonary function, her clinical symptoms, which included dyspnea and chylothorax, were significantly improved after treatment with sirolimus. Although a beneficial effect of sirolimus in the treatment of LAM has not been definitively determined, this report may provide useful information for management of recurrent LAM after lung transplantation.
在本研究中，我们报告了一例28岁女性患者，她在接受双侧活体供体肺叶移植后，同种异体移植物中出现复发性淋巴管平滑肌瘤病（LAM）。尽管她在接受他克莫司和泼尼松龙免疫抑制治疗后的术后过程平稳，未发生排斥反应，但在移植后5年，她出现了呼吸急促以及进行性乳糜性胸腔积液，双肺出现弥漫性囊性改变。尽管根据影像学检查结果和肺功能恶化诊断为LAM复发，但在接受西罗莫司治疗后，她包括呼吸困难和乳糜胸在内的临床症状得到了显著改善。虽然西罗莫司治疗LAM的有益效果尚未最终确定，但本报告可能为肺移植后复发性LAM的管理提供有用信息。
