Alvarez Ofelia A, Kjellin Ingrid, Zuppan Craig W
Department of Pediatrics, University of Miami, Miami, Florida 33101,USA.
J Pediatr Hematol Oncol. 2004 Feb;26(2):136-41. doi: 10.1097/00043426-200402000-00018.
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.
一名8岁男孩出现纵隔肿块、肺部浸润和弥散性血管内凝血，被诊断为淋巴管瘤病。尽管接受了药物治疗，但他仍出现多器官功能衰竭并死亡。作者讨论了诊断结果、药物治疗和病理学，并回顾了文献中另外52例胸段淋巴管瘤病病例。患者的表现包括乳糜胸（49%）、肿块（47%）、肺部浸润（45%）、骨病变（39%）、脾病变（19%）、颈部受累（15%）、弥散性血管内凝血（9%）和皮肤受累（7%）。儿童（<16岁）的预后比年长患者差（死亡率分别为39%和0%）。所有死亡患者均有肺实质受累或胸腔积液。胸段淋巴管瘤病应纳入伴有肺部表现的纵隔肿块的鉴别诊断。
