Abstract: Lymphangiomatosis is a rare disease， which is characterized by abnormal proliferation of lymphatic endothelial cells. The proliferative lesions are usually diffuse and multicentric in lymphangiomatosis. It is referred to as diffuse pulmonary lymphangiomatosis when the thoracic viscera including lung， mediastinum and pleura are involved. Usually， diffuse pulmonary lymphangiomatosis has poor  prognosis. Recently， the understanding of the mechanisms of diffuse pulmonary lymphangiomatosis has been impoved by the breakthough of genetics as well as cellular?signaling pathway research. What’s more， the clinical application of targeted drug and their significant effects have brought a new hope to the treatment of diffuse pulmonary lymphangiomatosis. This article mainly introduces the pathogenesis， clinical manifestations， diagnostic methods and treatment progress of diffuse pulmonary lymphangiomatosis in children.

Key words: lymphangiomatosis, lymphatic malformation, generalized lymphatic anomaly, chylothorax, targeted drug

摘要： 淋巴管瘤病是一种罕见的淋巴管异常增生性疾病。病变通常呈弥漫性、多灶性分布，当累及肺、纵隔、胸膜等胸腔内器官及组织时，也称为弥漫性肺淋巴管瘤病。通常认为，弥漫性肺淋巴管瘤病预后欠佳。近年来，随着遗传学以及细胞信号通路研究领域的突破，对弥漫性肺淋巴管瘤病的发病机制有了新的认识。而靶向药物的应用及展现出来的良好疗效，也为弥漫性肺淋巴管瘤病的治疗带来了新希望。该文着重介绍了儿童弥漫性肺淋巴管瘤病的发病机制、临床表现、诊断方法和治疗进展等。

关键词: 淋巴管瘤病, 淋巴管畸形, 泛发性淋巴管异常, 乳糜胸, 靶向药物