Miller J G, Orton C I
Department of Plastic and Reconstructive Surgery, Withington Hospital, Manchester.
Br J Plast Surg. 1992 Oct;45(7):559-61. doi: 10.1016/0007-1226(92)90157-s.
Kasabach-Merritt syndrome is the association of thrombocytopenia, spontaneous bleeding, and enlargement of a haemangioma. It is caused by an intense, self-perpetuating process of clot-formation and lysis within the abnormal vascular channels of the haemangioma, and results in consumption of platelets and clotting factors. Treatment involves ablation of the lesion with or without pharmacological manipulation of the coagulation and fibrinolytic systems. No single therapeutic modality is universally successful but a combination of radiotherapy and corticosteroids can result in a dramatic, immediate response with minimal long term complications.
卡萨巴赫-梅里特综合征是一种伴有血小板减少、自发性出血和血管瘤增大的病症。它是由血管瘤异常血管通道内强烈的、自我持续的凝血和纤溶过程引起的,会导致血小板和凝血因子的消耗。治疗方法包括对病变进行消融,可联合或不联合对凝血和纤溶系统进行药物干预。没有一种单一的治疗方式能普遍取得成功,但放疗和皮质类固醇联合使用可产生显著的即时反应,且长期并发症最少。