Premium
Orthotopic liver transplantation in a patient with combined hemophilia A and B
Author(s) -
Delorme Michael A.,
Adams Paul C.,
Grant David,
Ghent Cameron N.,
Walker Irwin R.,
Wall William J.
Publication year - 1990
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830330211
Subject(s) - medicine , cirrhosis , coagulopathy , factor ix , liver transplantation , fresh frozen plasma , perioperative , liver disease , hemostasis , transplantation , surgery , factor vii , gastroenterology , hepatitis b , prothrombin time , prothrombin complex concentrate , coagulation , platelet , warfarin , atrial fibrillation
A 38‐year‐old man with severe factor IX and mild factor VIII deficiencies complicated by cirrhosis secondary to chronic non‐A non‐B hepatitis underwent orthotopic liver transplantation as treatment for both the cirrhosis and his congenital coagulopathy. Intraoperative hemostasis was obtained with factor VII‐depleted prothrombin complex concentrate and fresh frozen plasma. Factor VIII and factor IX levels were assayed frequently in the perioperative period, and both returned to normal within 24 hr and remained normal postoperatively. Liver transplantation can be considered as definitive therapy for hemophilia A and/or B with transfusion‐related liver disease.