Korean J Dermatol.
1995 Apr;33(2):363-368.
Successful Treatment of an Infant with Kasabach - Merritt Syndrome with Interferon Alfa - 2a
Abstract
- Kasabach- Merritt syndrame consists of thrombocytopenia, microangiopathic hemolytic anemia, and an acute or chronic censumptive coagulopathy in associati an with a rapidly enlarging hemangioma. Most vascular turrnrs causing Kasabach-Merritt syndon e are benign but the associated coagulopathy may be lifethreatening. Our patient, a 30-day-old female infant, was admitted after an increase in the size of a hemangioma present since birth on the right leg with extension to the lower abdomen and left inguinal area. Her herrnglobin count fell to 7.1g/dl, platelet count was 26,000/ul, prothrombin time was 26 seconds, and hypofibrinogenemia developed. The patient was treated with transfusions of platelets, fresh frozen plasma and red cells and prednisolone. for 2 weeks without improvement. We started interferon alfa-2a therapy. Over the past 6 months, he hemangioma progressively diminished in size withcut any serious complication.