title: [Kaposiform hemangioendothelioma associated with Kasabach-Merritt syndrome]
abstract: OBJECTIVE: To describe a case of kaposiform hemangioendothelioma, the only malignant tumor of vascular origin specific of childhood.METHODS: We report a case of a 40-days-old girl who presented with a giant hemangioma of the face. Rapid enlargement of the tumor lead to laryngeal compression with severe respiratory distress. She had also a consumptive thrombocytopenic coagulopathy (Kasabach-Merritt syndrome).RESULTS: She was admitted to the pediatric intensive care unit of the Hospital das Clínicas da Universidade Estadual de Campinas and mechanical ventilation was introduced. A ten day course of dexamethasone was only partially effective. We then started alpha-2a interferon at a dose of 1.8 million units/m(2)/day by subcutaneous route, but she died four days after the onset of this therapy. The microscopic features showed a kaposiform hemangioendothelioma.CONCLUSION: We discuss this unusual fatal evolution of a rapidly growing hemangioma and its hematological complications.
authors.affiliation: Hospital Materno-Infantil de Brasília, Brasília, DF, Brazil.
authors.forename: A P
authors.initials: AP
authors.lastname: Serafim
authors.forename: L C
authors.initials: LC
authors.lastname: Almeida Junior
authors.forename: M T
authors.initials: MT
authors.lastname: Silva
authors.forename: R B
authors.initials: RB
authors.lastname: Carvalho
authors.forename: A M
authors.initials: AM
authors.lastname: Altemani