Wednesday, January 7, 2009

Merit

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?

Image is licensed under Creative Commons Attributions 2.0 License.

5 comments:

Alex said...

dic?

CodeDog said...

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.

shabnam said...

Kasabach-Merritt Syndrome??? is that really a kaposiform hemangioendothelioma?

shabnam said...

i'm dying to know!

Craig Chen said...

well done! it's kasabach-merritt
-
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.