Sun Xiaopu, Lu Chengyu, Huang Zhanhang, Xu Jiaxing, Zhu Huiyi, Yang Sen, Chen Deihui
Department of Pediatrics, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong 510120, P.R. China.
Exp Ther Med. 2023 Mar 7;25(4):175. doi: 10.3892/etm.2023.11874. eCollection 2023 Apr.
The present study aimed to investigate the clinical characteristics of diffuse pulmonary lymphangioma (DPL) in children to improve the diagnosis and treatment of this disease. A case of pediatric DPL was observed for its clinical symptoms, imaging features, lung biopsy pathological characteristics and immunohistochemical phenotypes, and relevant literature was also reviewed. The main clinical manifestations of this pediatric patient were a cough, shortness of breath, hemoptysis, bloody chylothorax and pericardial effusion. Chest computed tomography showed a grid-like shadow and markedly thickened interlobular septa. Pathological examination revealed lymphatic vessel hyperplasia and expansion. Immunohistochemistry showed positive staining of lymphatic endothelial cells CD31 and D2-40. The patient's condition improved after combined treatment with methylprednisone, propranolol, sirolimus and somatostatin, whose bloody chylothorax also achieved good therapeutic effect after conservative treatment. Overall, the clinical and imaging appearances of DPL are lack of characterization, and its clinical manifestations include cough, shortness of breath and chylothorax. Computed tomography may show mesh-like shadows of both lungs and thickened interlobular septa. The definite diagnosis of DPL depends on biopsy pathology. In addition to this case, B-ultrasound-guided puncture biopsy is effective and safe, and propranolol-sirolimus treatment has a certain effect, but the clinical effect may be different. Conservative treatment of pleural effusion can result in better curative effect.
本研究旨在探讨儿童弥漫性肺淋巴管瘤（DPL）的临床特征，以提高该病的诊断和治疗水平。观察 1 例儿童 DPL 的临床症状、影像学特征、肺活检病理特征及免疫组化表型，并复习相关文献。该患儿的主要临床表现为咳嗽、气短、咯血、血性乳糜胸和心包积液。胸部计算机断层扫描显示网格状阴影及小叶间隔明显增厚。病理检查显示淋巴管增生和扩张。免疫组化显示淋巴管内皮细胞 CD31 和 D2-40 呈阳性染色。患儿经甲泼尼龙、普萘洛尔、西罗莫司和生长抑素联合治疗后病情好转，其血性乳糜胸经保守治疗也取得了良好疗效。总体而言，DPL 的临床和影像学表现缺乏特异性，其临床表现包括咳嗽、气短和乳糜胸。计算机断层扫描可能显示双肺网格状阴影及小叶间隔增厚。DPL 的确诊依赖于活检病理。除该病例外，B 超引导下穿刺活检有效且安全，普萘洛尔-西罗莫司治疗有一定效果，但临床效果可能存在差异。胸腔积液保守治疗可取得较好疗效。
