Liver Transplant For Budd-Chiari Syndrome Caused by Paroxysmal Nocturnal Hemoglobinuria

Abstract

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.