title: Hemophilia Liver Transplantation Observational Study.
abstract: Hepatitis C virus (HCV) infection is the leading cause of liver disease in hemophilia patients. In those with human immunodeficiency virus (HIV)/HCV coinfection, the rate of liver disease progression is greater than in HCV monoinfected individuals. Despite antiretroviral therapy, which slows HCV liver disease progression, some require transplantation. Whether transplant outcomes are worse in hemophilic (H) rather than nonhemophilic (NH) candidates is unknown. In order to determine rates and predictors of pretransplant and posttransplant survival, we conducted a retrospective observational study using United Network for Organ Sharing national transplant registry data, comparing HCV+ H and NH candidates. We identified 2502 HCV+ liver transplant candidates from 8 US university-based transplant centers, between January 1, 2004 to December 31, 2010, including 144 HIV+ (6%) and 2358 HIV-; 36 H (1%) and 2466 NH; 1213 (48%) transplanted and 1289 not transplanted. Other than male predominance and younger age, each were P < 0.001. Baseline data were comparable between H and NH. In univariate analysis, 90-day pretransplant mortality was associated with higher baseline Model for End-Stage Liver Disease (MELD; hazard ratio [HR] = 1.15; P < 0.001), lower baseline platelet count (HR = 0.9 per 25,000/µL; P = 0.04), and having HIV/HCV+ hemophilia (P = 0.003). In multivariate analysis, pretransplant mortality was associated with higher MELD (P < 0.001) and was significantly greater in HIV+ than HIV- groups (P = 0.001). However, it did not differ between HIV+ H and NH (HR = 1.7; P = 0.36). Among HIV/HCV+, posttransplant mortality was similar between H and NH, despite lower CD4 in H (P = 0.04). In conclusion, this observational study confirms that hemophilia per se does not have a specific influence on transplant outcomes and that HIV infection increases the risk of mortality in both H and NH patients. Liver Transplantation 23 762-768 2017 AASLD.
authors.affiliation: Division of Hematology/Oncology, University of Pittsburgh and Hemophilia Center of Western Pennsylvania, Pittsburgh, PA.
authors.forename: Margaret V
authors.initials: MV
authors.lastname: Ragni
authors.affiliation: Division of Transplant Surgery, Starzl Transplant Institute, University of Pittsburgh, Pittsburgh, PA.
authors.forename: Abhinav
authors.initials: A
authors.lastname: Humar
authors.affiliation: Division of Transplant Surgery, University of California, San Francisco, CA.
authors.forename: Peter G
authors.initials: PG
authors.lastname: Stock
authors.affiliation: Division of Infectious Diseases, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA.
authors.forename: Emily A
authors.initials: EA
authors.lastname: Blumberg
authors.affiliation: Transplant Center and Department of General Surgery, Cleveland Clinic Foundation, Cleveland, OH.
authors.forename: Bijan
authors.initials: B
authors.lastname: Eghtesad
authors.affiliation: Transplant Center and Department of General Surgery, Cleveland Clinic Foundation, Cleveland, OH.
authors.forename: John J
authors.initials: JJ
authors.lastname: Fung
authors.affiliation: Divisions of Infectious Diseases and Organ Transplantation, Northwestern University, Feinberg School of Medicine, Chicago, IL.
authors.forename: Valentina
authors.initials: V
authors.lastname: Stosor
authors.affiliation: Division of Transplant Surgery, Cedars-Sinai Medical Center, Los Angeles, CA.
authors.forename: Nicholas
authors.initials: N
authors.lastname: Nissen
authors.affiliation: Division of Transplant Medicine, Beth Israel Deaconess Medical Center, Boston, MA.
authors.forename: Michael T
authors.initials: MT
authors.lastname: Wong
authors.affiliation: Division of Digestive Disorders, University of Cincinnati, Cincinnati, OH.
authors.forename: Kenneth E
authors.initials: KE
authors.lastname: Sherman
authors.affiliation: Emmes Corporation, Baltimore, MD.
authors.forename: Donald M
authors.initials: DM
authors.lastname: Stablein
authors.affiliation: Emmes Corporation, Baltimore, MD.
authors.forename: Burc
authors.initials: B
authors.lastname: Barin
mesh_terms: D000328:Adult; D044966:Anti-Retroviral Agents / Q000627:therapeutic use; D015496:CD4-Positive T-Lymphocytes / Q000166:cytology; D060085:Coinfection / Q000401:mortality; D003627:Data Interpretation, Statistical; D018450:Disease Progression; D005260:Female; D015658:HIV Infections / Q000150:complications / Q000401:mortality / Q000601:surgery*; D006467:Hemophilia A / Q000150:complications / Q000401:mortality / Q000601:surgery*; D019698:Hepatitis C, Chronic / Q000150:complications / Q000401:mortality / Q000601:surgery*; D006801:Humans; D017093:Liver Failure / Q000150:complications / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D016017:Odds Ratio; D011183:Postoperative Complications; D012042:Registries; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D014481:United States
chemical_list: D044966:Anti-Retroviral Agents