[Unresectable giant hepatic hemangioma and Kasabach-Merritt syndrome].
The Kasabach-Merritt syndrome was first described in children with cutaneous hemangiomas, but it can exceptionally be associated with visceral hemangiomas, especially in adults. Clotting and fibrinolysis within the hemangioma are thought to cause the coagulopathy observed in the so-called Kasabach-Merritt syndrome. This localised form of intra-vascular coagulation can progress to a secondary increased systemic fibrinolysis with fatal outcome for 20 to 30% of the patients. A transient control of hematologic abnormalities can frequently be obtained with blood product support (platelets, fibrinogen, fresh plasma, cryoprecipitates) and heparinotherapy. But in the adult, the only radical alternative is surgical excision if technically feasible. We reported here the case of a 43 year-old woman with a giant unresectable hepatic hemangioma complicated with a Kasabach-Merritt syndrome.
About
I
I
Service de médecine interne A, hôpital de Foix, France.
Capdeville
J
J
Bernard
P
P
Lazorthes
F
F
Boneu
B
B
D000328:Adult; D004211:Disseminated Intravascular Coagulation / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D005260:Female; D006391:Hemangioma / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D013577:Syndrome
Hémangiome hépatique géant inextirpable et syndrome de Kasabach-Merritt.