Kasabach-Merrit syndrome in infants

Abstract

Methods: Four infants with Kasabach-Merrit syndrome syndrome have been treated at the University Hospital, Trabzon. They had large varied-site cutaneous hemangiomas. Diagnosis was performed with clinical and laboratory studies. All patients has severe thrombocytopenia and anemia. Fibrinogen and fibrin split products were examined in two patients and lower fibrinogen and over fibrin split products levels were detected in them. All patients were admitted to the intensive care unit and they were treated with antibiotics, fresh blood transfusion and thrombocyte suspension. Two out of four patients were previously treated with steroids unsuccessfully and one patient died due to disseminated intravascular coagulopathy. Three patients were treated with interferon alfa-2a and compression.

Results: In two patients the lesions regressed 60-80% following the five months therapy and in the other patient the lesion was completely excised after one month therapy.

Conclusions: Interferon alfa-2a and compression were found to be remarkably effective in the treatment of Kasabach-Merrit syndrome.