Treatment of Severe Coagulopathy in the Kasabach-Merritt Syndrome with Aminocaproic Acid and Cryoprecipitate

Abstract

The Kasabach-Merritt syndrome is characterized by thrombocytopenia associated with solitary or multiple hemangiomas.¹ Coagulation studies in patients with this disease frequently reveal a profile similar to that of disseminated intravascular coagulation.^{2 , 3} The disorder is of particular clinical interest since complete eradication of the tumor by surgical excision or radiation eliminates the coagulopathy.^{4 , 5} Although the syndrome is primarily a disease of infancy, some patients survive for long periods, particularly if the coagulation defect is mild. In the absence of treatment, fatal hemorrhage can result.^{4 5 6} The syndrome is unique in that the coagulopathy is believed to be a manifestation of clotting and . . .