NHÂN TRƯỜNG HỢP CHẨN ĐOÁN VÀ ĐIỀU TRỊ  LYMPHANGIOLEIOMATOMATOSIS (LAM)  TẠI BỆNH VIỆN QUÂN Y 175

Thị Anh Đào Phan; Thanh Tùng Trương; Thị Ngọc Hân Nguyễn

doi:10.59354/ydth175.2022.58

Keywords:

Lymphangioleiomatomatosis(LAM)

Abstract

Lymphangioleiomatomatosis (LAM) is a multisystem disorder characterized by the growth of smooth muscle cells around the lungs, pulmonary blood vessels, lymphatic vessels, and pleura leading to the formation of cystic structure in the lung parenchyma and other manifestations such as angioma and lymphoma. According to other reports, this is a rare and fatal disease in young women. In this report, we describe a 33 years old woman with a history of reoccurrent pneumothorax, hospitalized due to chest pain, acute dyspnea. Chest X-ray and thoracic computed tomography images showed multicystic lung lesions, diffuse interstitial fibrosis, bilateral pneumothorax, and finally diagnosis of LAM has been confirmed. Our patient is no longer suitable for treatment with mTOR inhibitors (sirolimus) and treatment in an institute where lung transplant is available is recommended

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