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.

• Authors: Suleyman Yedibela, Werner Hohenberger
• Journal: Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation (Exp Clin Transplant) Vol. 6 Issue 3 Pg. 180-3 (Sep 2008) ISSN: 1304-0855 [Print] Turkey
• PMID: 18954293 (Publication Type: Case Reports, Journal Article)

Topics

• Budd-Chiari Syndrome (etiology, surgery)
• Follow-Up Studies
• Hemoglobinuria, Paroxysmal (complications)
• Humans
• Liver Transplantation
• Male
• Treatment Outcome
• Young Adult