The Kasabach-Merritt phenomenon may be seen in infants and children with certain types of vascular tumors. This is a consumptive coagulopathy which has a significant morbidity and mortality.


Features of Kasabach-Merritt phenomenon:

(1) thrombocytopenia (usually < 60,000 per µL)

(2) hypofibrinogenemia

(3) elevated D-dimer (fibrin split products)

(4) PT and PTT may be normal

(5) clinical coagulopathy (purpura, hemorrhage, etc.)


Tumors associated with the phenomena:

(1) Kaposiform hemangioendothelioma

(2) tufted angioma


Tumors not associated with the phenomenon:

(1) infantile or juvenile hemangiomas (which often involute as the patient gets older)


Patients with other forms of vascular malformations may have evidence of a consumptive coagulopathy with low fibrinogen and elevated fibrin split products, but they do not develop the severe thrombocytopenia or severe morbidity seen in the Kasabach-Merritt phenomenon.


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