肺淋巴管平滑肌增生症是一種原因不明且罕見的疾病,幾乎都發生在停經前的婦女身上。其特徵為肺實質異常平滑肌細胞增生,造成肺功能逐漸惡化乃至死亡。肺外的病灶也可見於此病患者。 我們報告一位四十四歲的女性,其臨床表徵、放射學檢查和組織切片皆符合本病。同時在病人身上也發現腎臟及肝臟有血管肌肉脂肪瘤。此外,本病患的病理組織切片亦有一獨特的發現。我們回顧了相關的文獻並予以探討。
Pulmonary lymphangioleiomyomatosis (LAM), a rare disorder of unknown cause that occurs almost exclusively in women of childbearing years, and is characterized by a proliferation of abnormal smooth muscle cells within the lung parenchyma and elsewhere, leading to a progressive loss of lung function and death. We report a 44-year-old female presenting with the characteristic clinical, radiographic, and histologic features of LAM. In the examination, renal and hepatic angiomyolipoma (AML), and a unique histopathologic feature were found in this patient. The literature is reviewed for further discussion.
為了持續優化網站功能與使用者體驗,本網站將Cookies分析技術用於網站營運、分析和個人化服務之目的。
若您繼續瀏覽本網站,即表示您同意本網站使用Cookies。