Liver transplantation for sinusoidal obstructive syndrome (veno-occlusive disease): case report with review of the literature and the UNOS database.

Background: Severe sinusoidal obstructive syndrome (SOS) is a life-threatening complication of stem cell transplantation. We report the case of a young man transplanted for SOS.

Method: A single chart review with query of the United Network of Organ Sharing database and review of the medical literature.

Case: A 23-yr-old male diagnosed with chronic myeloid leukemia underwent a matched unrelated stem cell transplant. The conditioning regimen included high-dose cyclophosphamide and busulfan. Within one month, he developed painful hepatomegaly, jaundice, ascites, and weight gain, and was diagnosed with biopsy-proven SOS. Despite therapy with defibrotide, he continued to deteriorate with the development of progressive renal failure and encephalopathy. The patient underwent orthotopic liver transplantation. After surgery, he developed cytomegalovirus infection and six wk later presented with a bile leak, hepatic artery thrombosis, and a liver abscess. A repeat bone marrow biopsy showed no evidence of recurrent disease. Although the patient was listed for re-transplantation, he succumbed prior to an organ becoming available.

Conclusion: Severe SOS in the setting of bone marrow transplantation portends a poor prognosis. Careful patient selection, timing, and perhaps less immunosuppression should be considered when performing a liver transplantation in the setting of severe SOS.