A Case of Kasabach-Merritt Syndrome with Involvement of Airway Obstruction Treated with Interferon Alfa-2a / 대한소아혈액종양학회지

ABSTRACT

We have recently encounted a case of Kasabach-Merritt syndrome which were characterized by hemangioma, thrombocytopenia, and disseminated intravascular coagulation. The skin of anterior neck and tongue was involved by large mass of hemangioma. Patient was taken ventilator care from upper airway obstruction of hemangioma. He was placed on combined therapy with steroid and interferon alfa-2a for 13weeks. Blood platelet count increased to acceptable range(>20000/mm3) after 10weeks of interferon alfa-2a treatment and the hemangioma decreased in size. But, he expired from failure of airway maintenance, uncontrollable bleeding and septic shock on hospital day 93. A brief review of the literature ensues with the case report.