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Arch Surg. 2007;142(4):400.

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Answer: Kasabach-Merritt Syndrome in a Giant Cavernous Liver Hemangioma

Kasabach-Merritt syndrome (KMS) is characterized by severe thrombocytopenia secondary to platelet trapping by an abnormally proliferated endothelium within congenital subcutaneous and/or visceral hemangiomas. This is usually accompanied by a secondary consumptive coagulopathy, which is reflected by hypofibrinogenemia, elevated levels of fibrin degradation products, and fragmentation of red blood cells.¹ Neither the site nor the size of the hemangioma appears to reliably predict the occurrence of KMS, which is associated with a high mortality (30%-40%) as a result of uncontrollable bleeding secondary to disseminated intravascular coagulation.²

This syndrome has its highest incidence in early infancy and most of the cases are secondary to single or multiple subcutaneous hemangiomas.¹ However, those associated with a more severe phenotype are often localized in visceral organs, especially in the retroperitoneal space.³ The reported incidence of KMS in a giant hepatic hemangioma is very low and its occurrence in adults is exceptional.² A KMS secondary to . . . [Full Text of this Article]

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