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Published on: August 17, 2022
Liver transplantation for a pediatric patient with hemophilia B
Yukihiro Sanada1, Taizen Urahashi, Yoshiyuki Ihara
1Department of Transplant Surgery, Jichi Medical University, Tochigi, Japan. yuki371@jichi.ac.jp
Insights
This study details a successful pediatric living donor liver transplant (LDLT) for hemophilia B, using short-term factor replacement. The protocol involved factor IX concentrate infusions, ensuring a safe and uneventful recovery for the young patient.
Area of Science:
- Hepatology
- Hematology
- Transplantation
Background:
- Hemophilia B poses significant perioperative bleeding risks.
- Established protocols for factor replacement during liver transplantation (LT) are lacking.
- Pediatric liver transplantation in patients with bleeding disorders presents unique challenges.
Observation:
- A 4-year-old female with hemophilia B and an extrahepatic portosystemic shunt underwent a living donor liver transplant (LDLT).
- Perioperative management included short-term factor IX concentrate (Novact M) administration.
- Factor IX concentrate was given via bolus infusion at anesthesia induction, LDLT completion, and postoperatively for four days.
Findings:
- The patient received 80 U/kg of factor IX at induction and end of LDLT, followed by 40 U/kg on postoperative days 1-4.
- Postoperative factor IX plasma levels ranged from 34.5% to 64.9%.
- The patient experienced an uneventful recovery and remains well 2.5 years post-transplant.
Implications:
- This case demonstrates the feasibility and safety of a short-term factor replacement protocol for pediatric LDLT in hemophilia B patients.
- The described regimen can guide perioperative management for similar complex cases.
- Successful liver transplantation can effectively manage underlying bleeding disorders, improving long-term outcomes.
Abstract:
Hemophilia exposes patients to greater risks of bleeding complications during the perioperative period. However, there are no current protocols for factor replacement during LT. We herein describe a case of pediatric living donor LDLT performed for a patient with hemophilia B using perioperative short-term factor replacement. A 4-yr-old female patient with an extrahepatic portosystemic shunt and asymptomatic hemophilia B (factor IX activity 18.7%) underwent an ABO-compatible LDLT using a left lobe graft. The bleeding volume was 2980 mL. Freeze-dried human blood coagulation factor IX concentrate (Novact M, Kaketsuken, Japan) was administered at the induction of anesthesia and at the end of LDLT by bolus infusion (80 U/kg) and was continued by bolus infusion (40 U/kg) on POD 1, 2, 3, and 4. On POD 1, 5, 8, and 12, the factor IX plasma levels were 34.5%, 64.9%, 43.5%, and 53.1%, respectively. The postoperative course was uneventful, and the patient is currently doing well at 2.5 yr after LDLT. Factor concentrate should be administered at the induction of anesthesia and at the end of LT by bolus infusion, and thereafter be continued for a few days after LT by bolus infusion.
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