This lesion was at birth in this young child. Laboratory tests show a severe thrombocytopenia, hypofibrinogenemia, elevated fibrin degradation products, and a hemolytic anemia.Challenge: What's the diagnosis?
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dic?
Kasabach Merritt Syndrome
At first glance it looks like abdominal bleeding associated with hemophilia.
However, on closer examination it appears to be over vasculated tissue.
This points us to some type of Hemangioendothelioma.
With the thrombocytopenia, hypofibrinogenemia, and high fibrin degradation products it is a clasic presentation of hemangioma thrombocytopenia syndrome also called KMS.
Kasabach-Merritt Syndrome??? is that really a kaposiform hemangioendothelioma?
i'm dying to know!
well done! it's kasabach-merritt
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Merit
This is Kasabach-Merritt Syndrome, described in 1940 associated with a kaposiform hemangioendothelioma located in the trunk, arms and shoulder, lower extremity, and cervicofacial. It presents with the laboratory abnormalities described and is treated with corticosteroids, interferon-alpha, surgery, embolization, and chemotherapy.
Sources: UpToDate; Wikipedia.
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