Fujii Takayuki, Shimono Ryuichi, Tanaka Aya, Katami Hiroto
Department of Pediatric Surgery, Faculty of Medicine, Kagawa University, 1750-1 Ikenobe, Mikicho, Kitagun, Kagawa 761-0793, Japan.
Case Rep Pediatr. 2019 Apr 30;2019:3101357. doi: 10.1155/2019/3101357. eCollection 2019.
Generalized lymphatic anomaly (GLA) is characterized by diffuse or multicentric proliferation of dilated lymphatic vessels resembling common lymphatic malformation, and thoracic lesions can be related to a poor prognosis. Sirolimus, an inhibitor of the mammalian target of rapamycin, is effective against vascular anomalies with few severe adverse drug reactions. Here, we report the case of a patient with intractable hemothorax pleural effusion due to GLA who was treated with sirolimus and experienced disseminated intravascular coagulation. Although a standard treatment for GLA has not been established, pleural fluid might be reduced using the Kampo medicine Eppikajyutsuto.
泛发性淋巴管异常（GLA）的特征是扩张的淋巴管呈弥漫性或多中心性增殖，类似于常见的淋巴管畸形，且胸部病变可能与预后不良有关。西罗莫司是一种雷帕霉素靶蛋白抑制剂，对血管异常有效，且严重药物不良反应较少。在此，我们报告一例因GLA导致难治性血胸性胸腔积液的患者，该患者接受西罗莫司治疗后发生了弥散性血管内凝血。虽然尚未确立GLA的标准治疗方法，但使用汉方药物薏苡仁汤可能会减少胸腔积液。
