【摘 要】
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Objective Pulmonary lymphangioleiomyomatosis (PLAM) is a rare disease occurring exclusively in women, whose diagnosis is not easy, as it may have similar radiographic findings with other pulmonary dis
【机 构】
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Department of Respiratory and Critical Care Medicine,the First Affiliated Hospital of Xi'an Jiaoton
【出 处】
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2016中华医学会呼吸病学年会暨第十七次全国呼吸病学学术会议
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Objective Pulmonary lymphangioleiomyomatosis (PLAM) is a rare disease occurring exclusively in women, whose diagnosis is not easy, as it may have similar radiographic findings with other pulmonary disease. Methods In the present study, we reported a case of PLAM in a 46-year-old female in north-western China and reviewed the literature about PLAM. Results The female experienced dyspnea on exertion for one month.The chest CT revealed diffuse thin-walled cystic lesions, consistent with radiological features of PLAM.CT-guided lung biopsy showed that characteristic smooth muscle cells were positive for SMA, HMB45 and D2-40 by histology and immunohistochemistry (IHC) techniques, resulting in a diagnosis of PLAM.The patient had a hysterectomy for treating uterine myoma 12 years ago.Additionally, lung diffusion function was impaired slightly,abdominal CT showed a right renal cyst, and pelvic ultrasound exhibited a right oophoritic cyst.Sirolimus was administered, which improved the dyspnea and pulmonary function, and she still needs long term follow-up.Therefore, this is a rare case of PLAM with renal cyst, oophoritic cyst and uterine myoma in a female in north-western China. Conclusions PLAM is a rare proliferative disease in the lung.Chest CT characteristically showed that the cysts were bilateral, diffuse and thin-walled.Lung biopsy may provide evidence for the diagnosis of PLAM, which was treated with sirolimus.
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