title: Recent advances in the pathobiology and management of Kasabach-Merritt phenomenon.
abstract: Kasabach-Merritt Phenomenon (KMP) refers to the clinical constellation of thrombocytopenia, consumptive coagulopathy and purpura associated with Kaposiform haemangioedothelioma or tufted angioma, but not the more common infantile haemangioma. It shows a variable and unpredictable response to traditional pharmacological agents, such as steroids, vincristine or interferon alpha 2a or 2b. More recently, the interaction between platelets and endothelial cells and the proangiogenic phenotype that results has been recognized to underly the pathogenesis of this disorder. Recent efforts have attempted to target the platelet by using antiplatelet agents and by the withholding of platelet transfusions even in those patients who have significant thrombocytopenia and laboratory evidence of coagulopathy. Excellent response rates and prompt results have been achieved by combining antiplatelet therapy with vincristine, without the need for steroid use. This synergistic approach moves away from the conventional wisdom of treating the underlying lesion to control the coagulopathy. Sirolimus, which is directed against the PI3/AKT/mTOR downstream signalling pathway involved in lymphangiogenesis, has also shown promising results, although further study is needed.
authors.affiliation: Department of Haematology, Our Lady's Children's Hospital, Dublin, Ireland.
authors.forename: Ciara
authors.initials: C
authors.lastname: O'Rafferty
authors.affiliation: Department of Dermatology, Our Lady's Children's Hospital, Dublin, Ireland.
authors.forename: Grainne M
authors.initials: GM
authors.lastname: O'Regan
authors.affiliation: Department of Dermatology, Our Lady's Children's Hospital, Dublin, Ireland.|Trinity College, Dublin, Dublin, Ireland.
authors.forename: Alan D
authors.initials: AD
authors.lastname: Irvine
authors.affiliation: Department of Haematology, Our Lady's Children's Hospital, Dublin, Ireland.|Trinity College, Dublin, Dublin, Ireland.
authors.forename: Owen P
authors.initials: OP
authors.lastname: Smith
mesh_terms: D006801:Humans; D059885:Kasabach-Merritt Syndrome / Q000378:metabolism* / Q000503:physiopathology* / Q000628:therapy*; D015398:Signal Transduction; D013921:Thrombocytopenia / Q000473:pathology / Q000503:physiopathology / Q000628:therapy
keywords: Kaposiform haemangioendothelioma; KasabachMerritt Phenomenon; angiogenesis; thrombocytopenia; tufted angioma; vascular endothelial growth factor