Year 2018 / Volume 110 / Number 8
Case Report
Liver transplantation in hemophilia A and von Willebrand disease type 3: perioperative management and post-transplant outcome

522-526

DOI: 10.17235/reed.2018.5204/2017

Cristina Alonso Madrigal, Manuela Dobón Rebollo, Viviana Laredo de la Torre, Luis Palomera Bernal, Francisco Agustín García Gil,

Abstract
Introduction: infection with the hepatitis C virus (HCV) causes significant morbidity and mortality in patients with hemophilia. Finally, patients are considered for a liver transplantation (LT) due to cirrhosis and/or hepatocellular carcinoma (HCC). Case report: we report the cases of congenital coagulopathy and HCV infection that underwent LT in our institution. There were three patients with hemophilia A and one patient with von Willebrand disease (vWD) type 3. The coagulopathy outcome, perioperative management, factor and blood product usage and post-transplant survival were assessed. The deficient factor was initially administered in a direct bolus one hour before surgery with a target level of 100 IU/dl, which was sustained until stable hemostasis was reached. All three patients with hemophilia A were cured of their coagulopathy following transplantation. Factor VIII (FVIII) was 93 IU/dl at eleven years, 59 IU/dl at 13 months and 109 IU/dl at nine months post-transplant, in each case. The mean perioperative usage of FVIII concentrates was 175 IU/kg; concentrates were infused for an average of 36 hours post-transplant. The natural course of the bleeding symptoms of the patient with type-3 vWD was attenuated, with no detectable hemostatic levels of von Willebrand factor antigen (vWF:Ag) after transplantation. Discussion: after transplantation, hemophilia A cure and improved bleeding phenotype of type-3 vWD reduced morbidity and mortality. However, potential graft reinfection with HCV and relapsing HCC cast a shadow over these optimum results.
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20/06/2020 11:10:56
tengo hemofilia A leve y soy mujer muy buen artículo!


References
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Alonso Madrigal C, Dobón Rebollo M, Laredo de la Torre V, Palomera Bernal L, García Gil F. Liver transplantation in hemophilia A and von Willebrand disease type 3: perioperative management and post-transplant outcome. 5204/2017


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Publication history

Received: 30/07/2017

Accepted: 14/03/2018

Online First: 22/06/2018

Published: 30/07/2018

Article revision time: 192 days

Article Online First time: 327 days

Article editing time: 365 days


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