PB0231 - Liver Transplant Complicated by Reperfusion Injury in a Child with Severe Factor V Deficiency

Sunday, June 25, 2023

18:30 – 19:30 ET

Room: Exhibition

Presenting Author(s)

Mehul Thakkar, MD

Clinical Fellow, Hematology and Oncology
Department of Pediatrics, Washington University in St. Louis
RICHMOND HEIGHTS, Missouri, United States

Co-Author(s)

SS

Surbhi Saini

Assistant Professor of Pediatrics, Hematology and Oncology
Department of Pediatrics, Washington University in St. Louis
St. Louis, Missouri, United States
JS

Janis Stoll

Assistant Professor of Pediatrics, Gastroenterology, Hepatology and Nutrition
Department of Pediatrics, Washington University in St. Louis
St. Louis, Missouri, United States
SK

Sakil Kulkarni

Assistant Professor of Pediatrics, Gastroenterology, Hepatology and Nutrition
Department of Pediatrics, Washington University in St. Louis
St. Louis, Missouri, United States
DW

David Wilson

Professor of Pediatrics, Hematology and Oncology
Department of Pediatrics, Washington University in St. Louis
St. Louis, Missouri, United States
AK

Adeel Khan

Associate Professor of Surgery, Transplant Surgery
Department of Surgery, Washington University in St. Louis
St. Louis, Missouri, United States
MD

Maria Doyle

Professor of Surgery, Transplant Surgery
Department of Surgery, Washington University in St. Louis
St. Louis, Missouri, United States
MF

Melanie Fields

Assistant Professor of Pediatrics, Hematology and Oncology
Department of Pediatrics, Washington University in St. Louis
St. Louis, Missouri, United States

Background: Factor V (FV) deficiency is a rare autosomal recessive bleeding disorder impacting 1:1 million people.

Aims: We present the case of a 2-year-old male with severe FV deficiency that underwent a curative orthotopic liver transplant (OLT) complicated by reperfusion injury.

Methods: He initially presented at day of life 7 with bleeding from the umbilical stump, prolonged PT/INR and PTT, and a FV < 1% confirming the diagnosis of severe FV deficiency. NGS panel showed homozygosity for a p.Pro1187Argfs*175 mutation in the F5 gene. At 7 weeks of age, he presented with extensive intraparenchymal hemorrhage treated acutely with FFP to maintain FV activity >30% and twice weekly FFP infusions for bleeding prophylaxis thereafter. This case report details his course after being listed for OLT at 2 years of age

Results: A pre-operative pharmacokinetic study showed a peak FV of 15% after infusion of 10ml/kg FFP and an estimated half-life of 12 hours (Figure 1). Pre-operatively, he received 20 ml/kg of FFP and 10 mL/kg of platelets. Intra-operatively, FFP was transfused at 1.5 mL/kg/hr until anastomosis. His postoperative course was complicated by ischemic reperfusion injury with peak AST >7000 U/L, ALT 3162 U/L, and direct bilirubin 5.1 mg/dL 40 hours post-transplant. His post-operative FV nadir was 8%. He required 10 ml/kg of FFP 12 hours post-transplant with normalization of his FV to 55% on POD 4 (Figure 2).

Conclusion(s): In contrast to the first two reports of a curative liver transplant for FV deficiency where FV normalized within 24 hours of transplant, this case suggests postoperative coagulation support may be required in the setting of reperfusion injury.