NEWBORNS WITH KASABACH-MERRITT PHENOMENON-ASSOCIATED KAPOSIFORM HEMANGIOENDOTHELIOMA: A REPORT OF 6 CASES

Abstract

OBJECTIVE: Combination of kaposiform hemangioendothelioma (KHE) and Kasabach-Merritt phenomenon (KMP) in newborn children is a life-threatening constellation. The purpose of the study is the choice of the diagnostic and treatment methods in these patients and evaluating the effectiveness of treatment using radiological methods of investigation. The study enrolled 6 newborn patients with KHE within a period 2013 - 2018. MRI (CT) performed to make the diagnosis and evaluate treatment response. Hypervascular mass accompanied by reticular lymphedema, hyper intensive in T2 WI; isointensive in T1 WI, intense contrast enhancement, heterogeneous diffusion restriction were unique MRI characteristics of KHE. The sustained remission was achieved with treatment by propranolol (n=2), vincristine (n=1), and their combination (n=3).