Successful treatment with interferon-alpha of Kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon in infancy

Abstract

Kaposiform hemangioendothelioma (KHE), a rare vascular neoplasm that mainly occurs during infancy, is often associated with the Kasabach-Merritt phenomenon (KMP) characterized by consumptive coagulopathy and thrombocytopenia. Although the systemic administration of corticosteroids is often used as a first-line therapy, there exist refractory cases that require alternative treatment. Here, we report the case of an infant with KHE spreading from the oral cavity to the anterior chest wall, who developed KMP and respiratory failure due to airway obstruction. The tumor was refractory to various treatments, but responded to treatment with interferon-alpha (IFNα). Despite a concern regarding its side effects in infants, IFNα can be included as a treatment option for refractory KHE.