Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon in an infant: case report and review of the literature

Abstract

Kaposiform hemangioendothelioma is an endothelial-derived cell neoplasm, with a variable clinical spectrum between benign and aggressive, rare in childhood and adolescence, associated with the Kasabach-Merritt phenomenon, which is a coagulopathy that can be fatal, and therefore requires a diagnosis and timely treatment.The case of a 2-month-old female patient is described, with a dark red vascular lesion in the proximal region of the middle third of the left leg, non-depressible with poor response to initial drug treatment and sclerotherapy. In addition, paraclinical findings compatible with the Kasabach-Merritt phenomenon requiring multiple transfusions. A biopsy of the lesion compatible with kaposiform hemangioendothelioma was performed, continuing treatment with vincristine, steroids and propranolol with adequate clinical evolution.