New recommendations for Kaposiform hemangioendothelioma

Abstract

Many of us remember hearing about Kasabach-Merritt phenomenon (KMP) on rounds as an intern in the nursery and conjured visions of large hemangiomas requiring multiple units of platelets to combat a consumptive coagulopathy. In this issue of The Journal, Drolet et al report from a National Institutes of Health consensus working group on kaposiform hemangioendothelimoma (KHE), a rare vascular tumor typically presenting in infancy at the neck, axilla, or groin, as firm, solitary, >5 cm tumors, previously lumped together with infantile hemangioma. KHE is synonymous with tufted angioma, which was described in the late 1980s. KHE presents often with KMP, defined as consumptive platelet coagulopathy with hypofibrinogemia and elevated markers of coagulation activation. Recommendations for unresectable KHE, with or without KMP, are combined intravenous vincristine plus intravenous methylprednisolone or oral prednisolone with KMP, and oral prednisolone alone without KMP. Platelet transfusion for KMP is indicated only prior to surgery or for active bleeding. Interns can sleep better. Article page 285▶ Consensus-Derived Practice Standards Plan for Complicated Kaposiform HemangioendotheliomaThe Journal of PediatricsVol. 163Issue 1PreviewKaposiform hemangioendothelioma (KHE) is a rare, potentially life-threatening vascular tumor often associated with a coagulopathy known as Kasabach–Merritt phenomenon (KMP).1 Optimal therapy for KHE is not known, and despite well-published classification systems, physicians still confuse this entity with other vascular anomalies. In the interest of standardizing clinical practice across specialties and institutions and to establish a basis for the design of comparative effectiveness studies in vascular tumors, we have developed a consensus-derived standard of practice for the treatment of KHE with and without associated KMP. Full-Text PDF