论文标题
由Enteroatmospheric瘘产生的Kasabach-Merritt综合征
Kasabach-Merritt syndrome arising from an Enteroatmospheric Fistula
论文作者
论文摘要
Kasabach-Merritt综合征（KMS）是一种罕见的，威胁生命的疾病，其特征在于深度血小板减少症，低纤维蛋白原血症，部分血小板质质蛋白时间升高，也可能与微血管病性溶血性贫血有关。众所周知，这种现象与血管状血管内皮瘤和簇绒血管瘤的血管状肿瘤显着相关。然而，最近的文献表明它在各种血管畸形的环境中（即没有内皮细胞的肿瘤增殖）的存在。该报告的重点是在生命的第一年中患者，他在高度血管EnterAtotospheric瘘管的情况下经历了长期消耗性的凝血病。与上述综合征共享许多特征，这种异常表明，卡萨巴赫 - 梅里特现象与独特的血管畸形的新型关联。尽管可能致命，但卡萨巴赫 - 梅里特综合症可以通过适当的诊断和管理解决。揭示新的协会可以帮助改善以后的情况。
Kasabach-Merritt syndrome (KMS) is a rare, life-threatening condition that is characterized by profound thrombocytopenia, hypofibrinogenemia, elevated partial thromboplastin time, and may also be associated with microangiopathic hemolytic anemia. It is well established that this phenomenon is notably associated with the vascular tumors kaposiform hemangioendothelioma and tufted angioma; however, recent literature has suggested its presence in the settings of various vascular malformations (i.e. without neoplastic proliferation of endothelial cells). This report focuses on a patient in the first year of life, who experienced a chronic, consumptive coagulopathy in the setting of a highly vascular enteroatmospheric fistula. Sharing many features with the aforementioned syndrome, this anomaly suggests a novel association of the Kasabach-Merritt phenomenon with a unique vascular malformation. Although potentially lethal, Kasabach-Merritt syndrome can resolve with appropriate diagnosis and management; uncovering new associations can help to improve recognition and treatment in future cases.
