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. Pediatr Blood Cancer 2007;49:852–854. © 2006 Wiley‐Liss, Inc.