Effective treatment of kaposiform hemangioendotheliomas associated with Kasabach-Merritt phenomenon using four-drug regimen

We report a case of a 6-month-old girl suffering from a kaposiform hemangioendothelioma of the chest wall, associated with Kasabach-Merritt phenomenon. Despite rapid intervention with cortisone and interferon alpha the tumor led to a life-threatening clinical condition with progressive growth and consumption coagulopathy under therapy. Because therapy for kaposiform hemangioendotheliomas with a single anti-angiogenic or anti-proliferative agent has not been reported to be very successful, we administered vincristine, combined with cyclophosphamide, actinomycin D, and methotrexate in a critically ill patient. After six cycles of the applied four-drug regiment, the infant was in remission, which has been maintained for 5 months since stopping therapy.