title: Kasabach-Merrit syndrome and adult hepatic epithelioid hemangioendothelioma an unusual association.
abstract: Kasabach-Merrit syndrome (KMS) is very rarely observed in adults associated with visceral hemangiomas. Hepatic epithelioid hemangioendothelioma (HEHE) is a very rare clinical entity with an intermediate malignant potential and a mortality rate of 20-30%. We described a case of KMS associated with HEHE in a 70 year old male patient who presented coagulation abnormalities, low platelet count and high fibrin degradation products that were typical features of KMS. Histopathology of the hepatic tissue and a positive immunostaining for endothelial markers confirmed the diagnosis of an HEHE. The first description of KMS was made in children in 1940. It was described as a "consumptive coagulopathy with capillary hemangiomas". However, recent reports have shown that this condition could be associated with kaposiform hemangioendotheliomas, an aggressive form of giant hemangioma. A giant hemangioma in adults as well as an hemangioendothelioma in children could be associated KMS, but an HEHE has been rarely reported in association with this syndrome in adult patients.
authors.affiliation: Division of Internal Medicine-Hepatology, Hospital General de Agudos Dr Cosme Argerich, Ciudad Autónoma de Buenos Aires, Alte Brown 240, (1155), Buenos Aires, Argentina. bernardo@frider.com.ar
authors.forename: Bernardo
authors.initials: B
authors.lastname: Frider
authors.forename: Andrés
authors.initials: A
authors.lastname: Bruno
authors.forename: Julio
authors.initials: J
authors.lastname: Selser
authors.forename: Rodríguez
authors.initials: R
authors.lastname: Vanesa
authors.forename: Pablo
authors.initials: P
authors.lastname: Pascual
authors.forename: Raquel
authors.initials: R
authors.lastname: Bistoletti
mesh_terms: D000368:Aged; D001706:Biopsy; D006390:Hemangioendothelioma / Q000150:complications / Q000473:pathology*; D006801:Humans; D008099:Liver / Q000473:pathology*; D008107:Liver Diseases / Q000150:complications / Q000473:pathology*; D008297:Male; D010976:Platelet Count; D013577:Syndrome