Revista espanola de enfermedades digestivas · 2018
Liver transplantation in hemophilia A and von Willebrand disease type 3: perioperative management and post-transplant outcome.
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Alonso Madrigal Cristina, Dobón Rebollo Manuela, Laredo de la Torre Viviana, Palomera Bernal Luis, García Gil Francisco Agustín
Аннотация
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.
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.