Successful Treatment of Sinusoidal Obstructive Syndrome with Deceased-Donor Liver Transplant Following Hematopoietic Stem Cell Transplant

Abstract

Sinusoidal obstructive syndrome, also known as venoocclusive disease, is a potentially life-threatening complication of hematopoietic stem cell transplant. The conditioning regimens given before hematopoietic stem cell transplant result in the production of toxic metabolites that trigger the activation, damage, and inflammation of the endothelial cells that line the sinusoids. Although liver transplant has been performed for treatment of sinusoidal obstructive syndrome, reports by various liver transplant centers worldwide have been limited and disappointing. We report our experience of successfully treating a patient with sinusoidal obstructive syndrome with deceased donor liver transplant. A 34-year-old male patient was referred to our department due to refractory ascites and jaundice within 1 month after receiving peripheral blood stem cell transplant for acute lymphocytic leukemia. He was diagnosed with biopsy-proven sinusoidal obstructive syndrome. Despite therapy with defibrotide, his condition continued to deteriorate, which included development of intractable pleural effusion, ascites, and progressive renal failure. After undergoing deceased donor liver transplant, the patient's serum bilirubin levels and prothrombin time began to decrease. The histology of the excised liver was compatible with hepatic sinusoidal obstructive syndrome. After being treated for pneumonia, pulmonary edema, and cytomegalovirus viremia, the patient was discharged 2 months after transplant. At 18 months after liver transplant, although he was treated for fungal pneumonia and is being considered for kidney transplant due to unsuccessful weaning from renal replacement therapy, the patient has shown stable liver function and no signs of either graft-versus-host disease or a relapse of acute lymphocytic leukemia. In conclusion, although severe sinusoidal obstructive syndrome in the setting of stem cell transplant has a poor prognosis, we suggest that deceased donor liver transplant should be considered in the treatment of select patients with life-threatening liver dysfunction after hematopoietic stem cell transplant.