单位：[1]China Japan Friendship Hosp, Ctr Resp Med, Dept Pulm & Crit Care Med, Beijing, Peoples R China[2]Natl Ctr Resp Med, Beijing, Peoples R China[3]Chinese Acad Med Sci, Inst Resp Med, Beijing, Peoples R China[4]Natl Clin Res Ctr Resp Dise, Beijing, Peoples R China[5]China Japan Friendship Hosp, Dept Pathol, Beijing, Peoples R China[6]China Japan Friendship Hosp, Dept Radiol, Beijing, Peoples R China
- Diffuse pulmonary lymphangiomatosis (DPL) is rare in adults. It is characterized by abnormal proliferation, dilatation, and thickening of the lymphatic channels in the lungs, pleura, and mediastinal soft tissue. Here, we report a case of DPL in a young adult man with recurrent productive cough. Chest computed tomography (CT) showed bilateral interlobular septal and peribronchovascular thickening and mediastinal soft tissue infiltration. Lung biopsy through video-assisted thoracic surgery demonstrated proliferation and dilatation of irregular lymphatic spaces, lined by flattened endothelial cells that were positive for CD31, D2-40, and factor VIII-related antigen on immunohistochemical staining. After treatment with propranolol for six months, the chest CT showed improved interlobular septal and peribronchovascular thickening and a unilateral pleural effusion, which turned bloody. Radiologic features can suggest the diagnosis of DPL. Surgical biopsy with adequate section size is critical in the diagnosis. Propranolol might be an effective and safe therapeutic option for patients with DPL.
基金：
CAMS Innovation Fund for Medical Sciences (CIFMS) [2020-I2M-CT-B-095]; National Key R&D Program of China, Ministry of Science and Technology of China [2018YFC1315100]; Elite Medical Pro-fessionals Project of China-Japan Friendship Hospital [ZRJY2021-QM11]
第一作者单位：[1]China Japan Friendship Hosp, Ctr Resp Med, Dept Pulm & Crit Care Med, Beijing, Peoples R China[2]Natl Ctr Resp Med, Beijing, Peoples R China[3]Chinese Acad Med Sci, Inst Resp Med, Beijing, Peoples R China[4]Natl Clin Res Ctr Resp Dise, Beijing, Peoples R China
通讯作者：
通讯机构：[1]China Japan Friendship Hosp, Ctr Resp Med, Dept Pulm & Crit Care Med, Beijing, Peoples R China[2]Natl Ctr Resp Med, Beijing, Peoples R China[3]Chinese Acad Med Sci, Inst Resp Med, Beijing, Peoples R China[4]Natl Clin Res Ctr Resp Dise, Beijing, Peoples R China[*1]Chinese Acad Med Sci, China Japan Friendship Hosp, Natl Clin Res Ctr Resp Dis,Natl Ctr Resp Med, Ctr Resp Med,Inst Resp Med,Dept Pulm & Crit Care, Beijing, Peoples R China
推荐引用方式(GB/T 7714)：
Zhang Shuai,Zhong Dingrong,Zhao Ling,et al.Diffuse pulmonary lymphangiomatosis involving lungs and mediastinal soft tissue[J].AMERICAN JOURNAL OF THE MEDICAL SCIENCES.2022,364(1):118-123.
APA：
Zhang, Shuai,Zhong, Dingrong,Zhao, Ling,Liu, Min,Gao, Qian...&Zhai, Zhenguo.(2022).Diffuse pulmonary lymphangiomatosis involving lungs and mediastinal soft tissue.AMERICAN JOURNAL OF THE MEDICAL SCIENCES,364,(1)
MLA：
Zhang, Shuai,et al."Diffuse pulmonary lymphangiomatosis involving lungs and mediastinal soft tissue".AMERICAN JOURNAL OF THE MEDICAL SCIENCES 364..1(2022):118-123
