Liver transplant for Budd-Chiari syndrome caused by paroxysmal nocturnal hemoglobinuria
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Updated: Jun 28, 2026
Technique of Porcine Liver Procurement and Orthotopic Transplantation using an Active Porto-Caval Shunt
Published on: May 7, 2015
Suleyman Yedibela1, Werner Hohenberger
1Department of Visceral, Vascular, Thoracic Surgery, and Transplantation, University of Leipzig, Liebigstr.20, D-04103 Leipzig, Germany. sueleyman.yedibela@medizin.uni-leipzig.de
Objectives:
Paroxysmal nocturnal hemoglobinuria is a rare acquired nocturnal disorder of the hematopoietic stem cells. The major causes of associated morbidity and mortality are chronic intravascular hemolysis, pancytopenia, and venous thrombosis.
Patients:
We report on a 20-year-old man with advanced Budd-Chiari syndrome caused by paroxysmal nocturnal hemoglobinuria, who underwent an emergency liver transplant.
Results:
At the time of this writing, the patient has good primary hepatic function, and, although not receiving specific medication, shows no signs of pancytopenia. Anticoagulation with low-dose acetylsalicylic acid was initiated. Forty-eight months after the transplant, there are no signs of thromboembolic complications affecting the liver.
Conclusions:
Liver transplant is an appropriate treatment for Budd-Chiari syndrome caused by paroxysmal nocturnal hemoglobinuria. Supplemented by long-term low-dose anticoagulation treatment, liver transplant is superior to other surgical options, particularly when liver disease is advanced.
