Is Liver Transplantation a Cure for Afibrinogenemia?

Abstract Background: Congenital afibrinogenemia is a rare coagulation disorder characterized by the absence of fibrinogen, leading to both bleeding and paradoxical thrombotic complications. Liver transplantation has been proposed as a potential curative approach, particularly in patients with concomitant severe liver disease. This report aims to present a case of afibrinogenemia undergoing liver transplantation and to discuss the associated clinical challenges. Case description: A 43-year-old male with known congenital afibrinogenemia and chronic liver disease secondary to thrombosis underwent liver transplantation in 2019. He later presented with abdominal distension, pain, nausea, and vomiting. Imaging revealed portal vein thrombosis and stent occlusion. The patient developed decompensated liver disease characterized by ascites and grade 1 hepatic encephalopathy. Management included anticoagulation, antibiotics, and supportive therapy for encephalopathy and ascites. Due to vascular complications and clinical deterioration, he was referred to a specialized center for further interventional and transplant evaluation. Conclusion: Liver transplantation may offer a therapeutic option for patients with afibrinogenemia and advanced liver disease; however, it does not fully eliminate the risk of thrombotic complications. The management of these patients remains complex, particularly regarding anticoagulation and long-term follow-up. Clinical significance: This case highlights the persistent risk of thrombotic and hepatic complications after liver transplantation in patients with afibrinogenemia. It underscores the need for individualized management strategies and further research to establish optimal perioperative and postoperative protocols.