Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon: From Vincristine to Sirolimus

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The kaposiform hemangioendothelioma (KHE) is typically associated with thrombocytopenia and consumptive coagulopathy, which is called the Kasabach-Merritt phenomenon (KMP) (Enjolras O et al., J Pediatr 1997; 130: 631–664). The aim of therapy is to shrink the tumor, thereby alleviating the coagulopathy. Proven medications are above all prednisolone or vincristine, and, as recently reported, the mTOR-inhibitor (inhibitor of mammalian target of rapamycin) sirolimus (Blatt J et al., Pediatr Blood Cancer 2010; 55: 1396–1398). We here describe our management of 2 neonates in whom KHE was associated with severe consumptive coagulopathy.