Browsing by Author "Yedibela, Süleyman"

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    Liver Transplant For Budd-Chiari Syndrome Caused by Paroxysmal Nocturnal Hemoglobinuria
    (Başkent Üniversitesi, 2008-09) Yedibela, Süleyman; Hohenberger, Werner
    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.