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Treatment of refractory hemorrhage with factor XIII in a patient with hemophilia A with inhibitor
Authors:Christopher Ng MD  Christopher C Silliman MD  PhD  Gabrielle Pearl BS  MLS  Whitney Smith MT SH  Marilyn Manco‐Johnson MD  Michael Wang MD
Institution:1. Center for Cancer and Blood Disorders, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado;2. Research Department, Bonfils Blood Center, Denver, Colorado;3. Department of Pathology and Laboratory Medicine, Children's Hospital Colorado, Aurora, Colorado;4. Hemophilia and Thrombosis Center, University of Colorado School of Medicine, Aurora, Colorado
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. Pediatr Blood Cancer 2013; 60: E23–E25. © 2013 Wiley Periodicals, Inc.
Keywords:bypassing agents  FXIII  hemophilia  inhibitor  refractory bleeding  thromboelastography
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