ABSTRACT
Kasabach-Merritt phenomenon (KMP) is a life-threatening situation in the pediatric age group. It is characterized by thrombocytopenic consumptive coagulopathy. KMP is almost always associated with two uncommon vascular tumors, kaposiform hemangioendotheliomas (KHEs) and tufted angiomas (TAs). Early recognition and intervention are important. Hemodynamic stability should be achieved as a first step of the management with the use of fresh frozen plasma infusions. Surgical excision and embolization, when feasible, are therapeutic. Oral prednisolone with or without vincristine has been successfully used as first-line medical management. Oral sirolimus has shown its efficacy and safety in various case series. Propranolol and antiplatelet drugs are commonly used as a second-line therapy.
