Introduction: Hepatic sinusoidal obstruction syndrome (SOS), previously known as veno-occlusive disease, is a rare syndrome that typically occurs after hematopoietic cell transplantation and is caused by injury to sinusoidal endothelial cells, which activates an inflammatory response with involvement of coagulation and fibrinolytic pathways, ultimately causing liver necrosis. Risk factors for SOS include aggressive myeloablative regimens. Treatment includes defibrotide and supportive management for severe disease. Transjugular intrahepatic portosystemic shunt placement and liver transplant are rarely indicated for patients in the setting of failed medical therapy with only case reports described in the literature. We describe a case of a patient with SOS who underwent liver transplantation in order to add to the medical literature and help inform future care teams.
Case Description/Methods: A 46-year-old male with medical history significant for acute myeloid leukemia status post allogenic stem cell transplant presented with abdominal pain, diarrhea, and nausea and vomiting. Labs were notable for transaminases in the thousands with transjugular biopsy showing peliosis hepatitis, prominent centrilobular sinusoidal dilation, and focal fibrous obliteration of small venules, consistent with SOS. Hospitalization was also complicated by hypoxic respiratory failure requiring intubation for airway protection secondary to encephalopathy and renal failure. Patient was started on defibrotide but liver function continued to worsen. He ultimately underwent orthotopic liver transplantation but remained persistently hypotensive despite multiple vasopressors with evidence of shock liver. Patient passed away despite maximal medical therapies and orthotopic liver transplantation.
Discussion: Prognosis for patients with severe SOS is poor with patients developing irreversible liver disease and subsequent multiorgan failure despite medical therapies. Liver transplant is rarely done in attempts to stabilize patients with SOS. Our case describes a patient with acute myeloid leukemia status post allogenic stem cell transplant who presented with abdominal pain and was found to have fulminant liver failure from SOS. Patient passed away despite maximal medical therapies and orthotopic liver transplantation. Further research is warranted to assess whether OLT should be considered in patients who present with severe SOS.
