title: Correction of factor XI deficiency by liver transplantation.
abstract: Orthotopic liver transplantation for other diseases typically results in a coincidental cure for hemophilia A and B; however, long-term outcomes of liver transplant in hemophilia C are not very well described. Herein, the authors report a patient of severe congenital factor XI (FXI) deficiency who received an orthotopic liver transplant. The authors discuss the perioperative management and long-term outcomes. The normalization of his FXI levels confirms that the liver is the most clinically relevant site of synthesis of FXI.
authors.affiliation: aDivision of Hematology and Oncology bDivision of Laboratory Medicine and Pathology, -Jacksonville, Florida cDivision of Hematology and Oncology, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Eva
authors.initials: E
authors.lastname: Gupta
authors.forename: Laura
authors.initials: L
authors.lastname: Finn
authors.forename: Gretchen
authors.initials: G
authors.lastname: Johns
authors.forename: Rajiv K
authors.initials: RK
authors.lastname: Pruthi
authors.forename: Vivek
authors.initials: V
authors.lastname: Roy
mesh_terms: D019468:Disease Management; D005172:Factor XI / Q000096:biosynthesis*; D005173:Factor XI Deficiency / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008099:Liver / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D008875:Middle Aged; D014184:Transplantation, Homologous; D016896:Treatment Outcome
chemical_list: D005172:Factor XI