MULTIFARIOUS THERAPEUTIC APPROACH IN THE KASABACH-MERRITT SYNDROME

The Kasabach-Merritt syndrome is a rare occurence in newborns characte rized by thrombocytopaenia, consumption coagulopathy and sometimes, mi croangiopathic hemolytic anemia following gigantic haemangiomas. This syndrome, if not properly treated presents mortality rare. Till now no specific therapeutic protocols has been to manage this unfrequent dis ease, but in the last decades cortico-therapy was the most common trea tment, even if it proved to be effective only in case of large skin he mangiomas without coagulopathy. At the same time the use of radiothera py was reduced because of complications as scar formation and secondar y neoplasias. This paper describes a case presenting some peculiar dia gnostic difficulties who was treated with a multifarious therapeutic a pproach associated to reduce the hemangioma size and control the coagu lopathy. At birth, the infant showed severe anemia and thrombocytopeni a without clear cutaneous signs of a vascular lesion. Evidence occurre d only after some time and at that moment echodoppler and MR confirmed the diagnosis of Kasabach-Merritt syndrome. The hemangioma occuped th e whole right hemitorax. Initial treatment with platelet and clotting factors replacement was quickly associated with glucocorticoids (betam etasone 0.2 mg/Kg/day for 4 weeks). The haemangioma stopped growing, b ut coagulopathy and bleeding persisted. The patient was then subjected to low dose radiotherapy (100 + 100 + 150 cGy in two months), compare d to the literature (2 to 5 times as low). Cortico-therapy was maintai ned in association with epsilon-aminocaproic acid administration. Resu lts were monitored with periodical MR examinations that showed a progr essive improvement a month after the first radiotherapy administration . This case has proved that the Kasabach-Merritt syndrome can be treat ed with significant results using several approaches. In our experienc e, the following three main steps were successful: first a symptomatic therapy with blood transfusions, then the pharmacological approach an d, only in extremely unresponsive cases, radiotherapy at very low dose s.