Syndrome de Kasabach-Merrit compliquant un hémangiome géant du foie

Journal Africain d?Hépato-Gastroentérologie 06/2011; 5(2):119-122. DOI: 10.1007/s12157-011-0253-7


BackgroundGiant hepatic hemangiomas are very rare. These hemangiomas can be complicated exceptionally by the Kasabach-Meritt syndrome.
This syndrome associates a coagulopathy and a hemolysis. We report a new case of a giant hepatic hemagioma associated with
the Kasabach-Merritt syndrome.

Case reportA 30-year-old-woman without medical history was admitted for right hypochondrium mass. Laboratory tests found pancytopenia,
and reduction in the activity of prothrombin and factor V. An ultrasonography and abdominal computed tomography scan disclosed
a giant hepatic mass pervading the whole peritoneum. Exploratory laparotomy revealed an hemangioma of almost the entirety
of the right liver. A right hepatectomy was realized. Histological examination confirmed the benign nature of the hemangioma.
The outcome was favorable, with regression of the Kasabach-Meritt syndrome.

Discussion and conclusionThe association of liver hemangioma and Kasabach-Merritt syndrome is uncommon. The physiopathology of this syndrome is still
not understood. The thrombocytopenia is due to a consumption of platelets within the lesion with the absence of coagulation
and fibrinolysis. Laboratory tests and imaging permit diagnosis. There is no consensus regarding the treatment which is surgical
when the hemangioma is pedunculated, complicated, or when the diagnosis is uncertain.

IntroductionLes hémangiomes hépatiques géants sont très rares et peuvent se compliquer exceptionnellement d’un syndrome de Kasabach-Meritt.
Ce syndrome traduit la survenue d’une coagulopathie de consommation au sein d’une tumeur vasculaire, associée à une thrombopénie
sévère. Nous en rapportons une nouvelle observation chez une jeune patiente de 30 ans.

ObservationUne patiente de 30 ans sans antécédents pathologiques notables était admise pour une masse de l’hypochondre droit. Le bilan
biologique montrait une pancytopénie, une diminution du taux de prothrombine et du facteur V. L’échographie et la tomodensitométrie
(TDM) abdominales ont montré une volumineuse masse hépatique envahissant la cavité péritonéale. Une laparotomie exploratrice
a révélé un angiome intéressant la quasi-totalité du foie droit. Une hépatectomie droite a été réalisée. L’examen anatomopathologique
a confirmé la nature bénigne de la tumeur. L’évolution était favorable avec régression du syndrome de Kasabach-Meritt.

Discussion et conclusionL’association d’un hémangiome hépatique au syndrome de Kasabach-Merritt reste rare. La physiopathologie de ce syndrome est
encore mal comprise. La thrombopénie est secondaire à une consommation intralésionnelle de plaquettes avec peu ou pas de coagulation
plasmatique et de fibrinolyse. Le diagnostic repose sur la biologie et l’imagerie. Il n’existe pas de consensus concernant
le traitement qui est chirurgical en cas d’hémangiome pédiculé, de complications ou de diagnostic incertain.

KeywordsGiant hepatic hemangioma–Kasabach-Meritt syndrome
Mots clésHémangiome hépatique géant–Syndrome de Kasabach-Meritt

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