title: Treatment of refractory hemorrhage with Factor XIII in a patient with hemophilia A with inhibitor.
abstract: An 11-year-old male with hemophilia A and a known high-titer Factor VIII inhibitor was admitted with retroperitoneal hemorrhage. The patient was receiving infusions of recombinant activated Factor VII (rFVIIa) for a recent elbow hemorrhage when retroperitoneal bleeding commenced. Despite increased dosing of rFVIIa and a dose of activated prothrombin complex concentrate (aPCC), he continued to hemorrhage and required several blood transfusions. Factor XIII was administered 1 hour after rFVIIa and the patient demonstrated cessation of bleeding and normalization of clot strength. Factor XIII may act as an adjuvant in effective clot stabilization in patients with hemophilia and inhibitory antibodies.
authors: Center for Cancer and Blood Disorders, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado 80045, USA.
authors: Christopher
authors: C
authors: Ng
authors: Christopher C
authors: CC
authors: Silliman
authors: Gabrielle
authors: G
authors: Pearl
authors: Whitney
authors: W
authors: Smith
authors: Marilyn
authors: M
authors: Manco-Johnson
authors: Michael
authors: M
authors: Wang
mesh_terms: D000906:Antibodies / Q000097:blood; D002648:Child; D003029:Coagulants / Q000627:therapeutic use*; D005167:Factor VII / Q000627:therapeutic use; D005169:Factor VIII / Q000276:immunology; D005176:Factor XIII / Q000627:therapeutic use*; D006467:Hemophilia A / Q000097:blood / Q000150:complications*; D006470:Hemorrhage / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D008297:Male
chemical_list: D000906:Antibodies; D003029:Coagulants; D005167:Factor VII; D005169:Factor VIII; D005176:Factor XIII