Kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon in an infant girl.

Kaposiform hemangioendothelioma (KHE) and tufted angioma are vascular benign tumors that can be associated with the rare condition Kasabach-Merritt Phenomenon (KMP). KMP is characterized by consumption coagulopathy with severe thrombocytopenia, hypofibrinogenemia, and elevated D-dimer. We here report successful treatment of a female infant with a mediastinal KHE where treatment was complicated by KMP, life-threatening infections, and pericardial effusion. Due to the absence of randomized clinical trials, there is currently no standardized treatment protocol available for KHE. In our case, the infant was treated successfully with prednisolone, vincristine, and sirolimus.