Introduction:

Kaposiform hemangioendothelioma is a rare, fast growing vascular tumor. Its main complication is the Kasabach-Merritt phenomenon, which is characterized by severe thrombocytopenia, consumption coagulation disorder, and purpura.

Case report:

We present a male newborn in whom it was detected that the left upper limb was very thickened from birth. Using magnetic resonance imaging, the diagnosis was reached. Management with steroids and chemotherapy was started, however the evolution was torpid and he died in the first week of life. The diagnosis was confirmed at autopsy.

Conclusions:

Patients with a rapidly growing vascular tumor should look for hematological alterations its extent in order to give timely treatment.

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