Ozeki Michio, Fujino Akihiro, Matsuoka Kentaro, Nosaka Shunsuke, Fukao Toshiyuki
Nihon Rinsho. 2015 Oct;73(10):1777-88.
Lymphangiomatosis (recently renamed "generalized lymphatic anomaly") is a rare disease of unknown etiology that features an increase in the number of lymphatic vessels in many different tissues. Gorham-Stout disease(GSD) is a related disease characterized by lymphatic vessels involving the bones and resulting in progressive bone destruction. Respective definitions remain unclear because these conditions largely overlap in the clinical setting and are both associated with pleural effusion and other visceral lesions. These two conditions have recently been differentiated based on imaging findings. GSD is characterized by progressive osteolysis with loss of cortical bone. These diseases present considerable diagnostic and therapeutic challenges. Implementation of basic and clinical research is mandatory to improve understanding of these conditions and optimize management.
淋巴管瘤病（最近更名为“全身性淋巴管异常”）是一种病因不明的罕见疾病，其特征是许多不同组织中的淋巴管数量增加。戈勒姆-斯托特病（GSD）是一种相关疾病，其特征是淋巴管累及骨骼并导致进行性骨质破坏。各自的定义仍不明确，因为这些病症在临床环境中大多重叠，并且都与胸腔积液和其他内脏病变相关。最近根据影像学检查结果对这两种病症进行了区分。GSD的特征是进行性骨质溶解伴皮质骨丧失。这些疾病带来了相当大的诊断和治疗挑战。开展基础和临床研究对于增进对这些病症的了解并优化管理是必不可少的。
