Kasabach-Merrit Syndrome with Congenital Hemangioma: A Case Report

Abstract

The most common paediatric neoplasm is haemangioma, which is a vascular lesion caused by excessive blood vessel proliferation. Haemangioma are congenital lesions that commonly affect newborns infants and children. They are generally benign and often resolve spontaneously. In 3 to 5% of cases, they can cause complications inherent to their size, to involvement of vital organs and to the concomitant coagulopathy like Kasabach-Merritt syndrome. Kasabach Meritt Syndrome (KMS) is a potentially fatal coagulopathy marked by an increasing haemangioma, extreme thrombocytopenia, and consumption coagulopathy. KMS is correlated with kaposiform hemangioendothelioma (KHE), tufted angiomas, and congenital haemangiomas in rare cases (CHs). Since Kasabach and Merritt identified the first case in 1940, nearly 200 cases have been published in the literature. More than 80% of cases develop within the first year of life. Early diagnosis and institution of treatment is associated with favorable outcome. Steroids are considered as the most effective 1st line treatment.