ABSTRACT
This syndrome, first described by Kasabach and Merritt in 1940,57 features platelet trapping by hemangiomas causing thrombocytopenia and consumptive coagulopathy. The sequestration of platelets in the hemangiomas in such cases has been confirmed by investigations demonstrating increased uptake of chromium51 tagged platelets within the hemangioma. It is now clear that most patients with the Kasabach-Merritt phenomenon do not have typical hemangiomas but instead have kaposiform hemangioendotheliomas or tufted angiomas.7 The exact trigger mechanism which initiates platelet trapping is not fully understood.24 Two-thirds of these cases present in the first 3 months of life during active growth of the lesion; only 12% of cases occur after 1 year of age.58 The mortality rate is high, at about 40%. Initial treatment consists of heparin, aspirin, fresh blood, platelet transfusion and steroids. If the infant becomes stable on this regime, surgical excision should be attempted. If surgery is not feasible, long-term treatment with steroids, compression therapy or embolization of the feeder vessels should be attempted.55
