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Klin Padiatr 2012; 224(06): 395-397
DOI: 10.1055/s-0032-1323823
DOI: 10.1055/s-0032-1323823
Short Communication
Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon: From Vincristine to Sirolimus
Kaposiformes Hämangioendotheliom mit Kasabach-Merritt Phänomen: Von Vincristin bis SirolimusFurther Information
Publication History
Publication Date:
15 October 2012 (online)
![](https://www.thieme-connect.de/media/klinpaed/201206/lookinside/thumbnails/10.1055-s-0032-1323823-1.jpg)
Background
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.