title: Successful treatment of Kasabach-Merritt syndrome with prednisone and epsilon-aminocaproic acid.
abstract: The Kasabach-Merritt syndrome is characterized by thrombocytopenia and localized coagulopathy associated with a hemangioma. Most techniques applied to eradicate the tumor or accelerate its involution (surgery, radiation therapy, embolization) are invasive and require transfusion of large amounts of blood products. In some cases, medical treatment is the only alternative. Efficacy of steroids and antifibronolytic agents has already been described, but even this approach is associated with the administration of blood products. We report two cases of infants with Kasabach-Merritt syndrome associated with cardiac and hepatic hemangiomas. At admission, both had signs of cardiac failure. They were successfully treated with prednisone and epsilon-aminocaproic acid (EACA). Blood products were not required once the diagnosis was made. These observations have important implications for the management of patients with Kasabach-Merritt syndrome because they show that even in severe cases blood transfusions can be avoided by the use of prednisone and EACA.
authors.affiliation: Hematology-Oncology, Hôpital Sainte-Justine, Montreal, Quebec, Canada.
authors.forename: M F
authors.initials: MF
authors.lastname: Dresse
authors.forename: M
authors.initials: M
authors.lastname: David
authors.forename: H
authors.initials: H
authors.lastname: Hume
authors.forename: H
authors.initials: H
authors.lastname: Blanchard
authors.forename: P
authors.initials: P
authors.lastname: Russo
authors.forename: N
authors.initials: N
authors.lastname: Van Doesberg
authors.forename: G E
authors.initials: GE
authors.lastname: Rivard
mesh_terms: D015119:Aminocaproic Acid / Q000627:therapeutic use*; D004211:Disseminated Intravascular Coagulation / Q000188:drug therapy*; D004359:Drug Therapy, Combination; D005260:Female; D006333:Heart Failure / Q000188:drug therapy; D006338:Heart Neoplasms / Q000188:drug therapy; D006391:Hemangioma / Q000188:drug therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008113:Liver Neoplasms / Q000188:drug therapy; D008297:Male; D011241:Prednisone / Q000627:therapeutic use*; D013577:Syndrome; D013921:Thrombocytopenia / Q000188:drug therapy*
chemical_list: D015119:Aminocaproic Acid; D011241:Prednisone