CAJ | 학술논문

目的：探讨淋巴管肌瘤病（Lymphangioleiomyomatosis，LAM）的临床特点。方法回顾分析一例肺淋巴管肌瘤病伴后腹膜淋巴管肌瘤病临床资料，结合文献，分析该病临床特点和诊治现状。结果本病好发于育龄期妇女，以喘气、乳糜胸、气胸、咯血等为主要表现，肺功能受损，肺外病变腹膜后常见；肺部CT为大小一致、分布均匀薄壁囊样改变，可伴液气胸，磨玻璃样改变；腹部CT常为后腹膜囊实性肿块伴淋巴结肿大。结论当患者具有相应呼吸道症状，且有典型的肺部及腹部CT表现时，考虑LAM，病检有特异性。
목적：탐토림파관기류병（Lymphangioleiomyomatosis，LAM）적림상특점。방법회고분석일례폐림파관기류병반후복막림파관기류병림상자료，결합문헌，분석해병림상특점화진치현상。결과본병호발우육령기부녀，이천기、유미흉、기흉、각혈등위주요표현，폐공능수손，폐외병변복막후상견；폐부CT위대소일치、분포균균박벽낭양개변，가반액기흉，마파리양개변；복부CT상위후복막낭실성종괴반림파결종대。결론당환자구유상응호흡도증상，차유전형적폐부급복부CT표현시，고필LAM，병검유특이성。
Objective To discuss the clinical features of Lymphangioleiomyomatosis.Methods One case of a pulmonary associated with retroperitoneal lymphangioleiomyomatosis was retrospectively analyzed. Analysis of clinical characteristic and the present situation of diagnosis and treatment of the disease was made combined with the literature.Results The disease mainly occurs in child-bearing women. The main clinical manifestations are pant, chylothorax, pneumothorax, and haemoptysis,etc. Lung function was impaired, retroperitoneal lesions were common extrapulmonary . CT showed thin-walled capsule sample change with same size and uniform distribution, liquid pneumothorax and grinding glass sample change can be associated. Abdominal CT demonstrated retroperitoneal cystic-solid tumors with lymph node enlargement.Conclusion LAM should be considered when patients have corresponding respiratory symptoms, accompanied with typical lung and abdominal CT performance. pathologic examination has speciifcity.