Ozeki Michio, Funato Michinori, Kanda Kaori, Ito Masahumi, Teramoto Takahide, Kaneko Hideo, Fukao Toshiyuki, Kondo Naomi
Department of Pediatrics, Gifu University Graduate School of Medicine, Gifu, Japan.
Pediatr Hematol Oncol. 2007 Oct-Nov;24(7):513-24. doi: 10.1080/08880010701533603.
Diffuse lymphangiomatosis is a very rare congenital disease, characterized by diffuse or multifocal lymphangioma in the skeletal tissue, spleen, liver, mediastinum, and/or lung. The prognosis is usually poor, especially for children with thoracic lesion, and treatments for the disease are controversial. The authors report a 9-year-old boy with diffuse lymphangiomatosis involving the thorax with pleural effusions, the spleen, and systemic bone. The patient was treated with pegylated interferon alfa-2b, and achieved good clinical and radiological improvement.
弥漫性淋巴管瘤病是一种非常罕见的先天性疾病,其特征是在骨骼组织、脾脏、肝脏、纵隔和/或肺部出现弥漫性或多灶性淋巴管瘤。预后通常较差,尤其是对于有胸部病变的儿童,并且该疾病的治疗存在争议。作者报告了一名9岁男孩,患有累及胸部伴胸腔积液、脾脏和全身骨骼的弥漫性淋巴管瘤病。该患者接受了聚乙二醇化干扰素α-2b治疗,并在临床和影像学上取得了良好的改善。