Angiosarcoma variant of Kasabach-Merritt syndrome.
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.
Alliot
C
C
Department of Haematology, University Hospital of Amiens, France.
Tribout
B
B
Barrios
M
M
Gontier
M F
MF
D000368:Aged; D001344:Autopsy; D001707:Biopsy, Needle; D003937:Diagnosis, Differential; D004211:Disseminated Intravascular Coagulation / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D004359:Drug Therapy, Combination; D017809:Fatal Outcome; D006394:Hemangiosarcoma / Q000150:complications / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008113:Liver Neoplasms / Q000150:complications / Q000473:pathology*; D008297:Male; D013577:Syndrome; D016896:Treatment Outcome