淋巴管瘤病
医学
弥漫性血管内凝血
乳糜胸
鉴别诊断
肺隔离症
胸腔穿刺术
胸腔积液
放射科
纵隔肿块
肺
淋巴系统
病理
内科学
作者
Ofelia Álvarez,Ingrid Kjellin,Craig W. Zuppan
出处
期刊:Journal of Pediatric Hematology Oncology
[Ovid Technologies (Wolters Kluwer)]
日期:2004-02-01
卷期号:26 (2): 136-141
被引量:75
标识
DOI:10.1097/00043426-200402000-00018
摘要
An 8-year-old boy who presented with a mediastinal mass, pulmonary infiltrates, and disseminated intravascular coagulation was diagnosed with lymphangiomatosis. Despite medical management, he developed multiple organ failure and died. The authors discuss the diagnostic findings, medical management, and pathology and review 52 additional cases of thoracic lymphangiomatosis from the literature. Patients presented with chylothorax (49%), a mass (47%), pulmonary infiltrates (45%), bone lesions (39%), splenic lesions (19%), cervical involvement (15%), disseminated intravascular coagulation (9%), and skin involvement (7%). Children (<16 years) had a worse prognosis than older patients (39% vs. 0% mortality). All patients who died had either parenchymal lung involvement or pleural effusion. Thoracic lymphangiomatosis should be included in the differential diagnosis of a mediastinal mass with pulmonary findings.
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