Sirolimus therapy for children with problematic Kaposiform haemangioendothelioma and tufted angioma

Abstract

Kaposiform haemangioendothelioma (KHE) and tufted angioma (TA) are rare vascular tumours that typically present during infancy or childhood. Originally regarded as distinct lesions, they are now considered as part of a spectrum. Presentation is usually as an enlarging, tender, blue or red mass. Both KHE and TA may be associated with Kasabach-Merritt Phenomenon (KMP), a severe haemorrhagic state arising from intralesional platelet trapping, microangiopathic haemolytic anaemia, and consumptive coagulopathy.

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