Kasabach-Merritt syndrome, typically associated with benign angiomatous tumors, can rarely be the presenting manifestation of a malignant hepatic angiosarcoma.
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KMS may herald hepatic angiosarcoma; extends the differential beyond benign tumors but leaves open broader implications from a single case.
Kasabach-Merritt syndrome is characterized by the occurrence of disseminated intravascular coagulation (DIC) usually caused by benign angiomatous tumours. Here we report the case of a 70-year-old man in whom DIC revealed a locally advanced hepatic tumour. Although DIC resolved with heparin, antithrombin III, fresh frozen plasma and corticosteroids, the patient died from haemoperitoneum following a fall, 3 months after the initial observation. Histopathological examination by autopsy allowed the diagnosis of hepatic angiosarcoma. The physiopathogenic mechanisms and treatment options are discussed.
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Alliot et al. (2001) studied this question.
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