Abstracto

Kasabach-Merrit syndrome with congenital hemangioma.

Archana V, Shafath Ahmed M, Sundari S

Haemangioma are vascular lesions resulting from abnormal proliferation of blood vessels they are the most common paediatric neoplasm. Haemangioma are congenital lesions that are common in new-borns infants and children. They are generally benign and often resolve spontaneously. However, 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 KasabachMerritt syndrome. Kasabach Meritt Syndrome (KMS) is a potentially life threatening coagulopathy characterized by enlarging haemangioma with severe thrombocytopenia and consumption coagulopathy. KMS is associated with kaposiform hemangioendothelioma (KHE), tufted angiomas and rarely with congenital haemangiomas (CHs). Almost 200 cases have been reported in the literature since Kasabach and Merritt described the first case in 1940. More than 80% of cases occur within the first year of life.