Kasabach-Merritt syndrome is a rare type of vascular tumor with aggressive behavior in association with thrombocytopenia and consumptive coagulopathy. A standard guideline has not been established to date. A 7-day-old male infant with Kasabach-Merritt syndrome arising from kaposiform hemangioendothelioma was successfully treated with systemic corticosteroid and surgery. Systemic corticosteroid including methylprednisolone was injected intravenously followed by an intralesional injection of compound betamethasone. This approach brought about an excellent response after the first treatment which was maintained long enough to provide us with an opportunity to excise the tumor. Systemic corticosteroid and surgery may be considered an option for Kasabach-Merritt syndrome, although well-designed studies are needed to quantify the benefits and risks of this treatment.
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