Orthotopic liver transplantation in a patient with combined hemophilia A and B.
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.
Delorme
M A
MA
Department of Medicine, University of Western Ontario, London, Canada.
Adams
P C
PC
Grant
D
D
Ghent
C N
CN
Walker
I R
IR
Wall
W J
WJ
D000293:Adolescent; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D001803:Blood Transfusion; D005164:Factor IX / Q000032:analysis; D005169:Factor VIII / Q000032:analysis; D006467:Hemophilia A / Q000097:blood / Q000150:complications / Q000628:therapy*; D002836:Hemophilia B / Q000097:blood / Q000150:complications / Q000628:therapy*; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D016031:Liver Transplantation; D008297:Male
D001779:Blood Coagulation Factors; C025667:prothrombin complex concentrates; D005169:Factor VIII; D005164:Factor IX; D006493:Heparin