Kasabach-Merritt 증후군의 수술적 치험례

Surgical Experience of the Kasabach-Merritt Syndrome

In 1940, Kasabach and Merritt first described the association of a large vascular tumor and thrombocytopenia and termed this Kasabach-Merritt(KM) syndrome. It is characterized by a rapidly enlarging vascular anomaly and consumptive coagulopathy with thrombocytopenia, prolonged prothrombin time and partial thromboplastin time, hypofibrinogenemia, and the presence of D-dimer and fibrin split product, with or without microangiopathic hemolytic anemia. This is a potentially life-threatening condition with mortality rates from 20 to 30% as a result of severe sepsis, coagulopathy, or invasion of vital organs. Treatment modalities are corticosteroids, interferon alfa-2a or 2b, chemotherapy(vincristine, cyclophosphamide, etc.), aspirin, dipyridamole, com- pression, radiation therapy, embolization of feeding vessels and surgical excision. A standard treatment regimen for KM syndrome has not been established and most reports on definitive management of these complex vascular lesions have been anecdotal, involving small numbers of patients. The authors have successfully treated a patient of KM syndrome with actively bleeding huge hemangioma by surgical excision. They present it with the review of articles.