S2726 No Biopsy Saves Life! Rare Incidence of Liver Transplantation for Unknown T-Cell Lymphoma-Induced Acute Liver Failure

Abstract

INTRODUCTION: Acute liver failure (ALF) is a rare condition characterized by acute hepatic injury causing rapid onset of coagulopathy and encephalopathy. Without prompt treatment, ALF carries an exceptionally high morbidity and mortality, and prognosis is particularly poor when ALF is attributed to malignant infiltration. We present a case of ALF of initially unknown etiology that required expedited liver transplantation. The patient was subsequently diagnosed with hepatic T-cell lymphoma, which is a potentially transplant disqualifying condition. Post-transplant, the patient completed chemotherapy and made a full recovery. CASE DESCRIPTION/METHODS: A 21-year-old male soldier without prior medical history was evacuated from an overseas deployment for 2 weeks of headaches, fatigue, nausea, and vomiting. Scleral icterus had developed in conjunction with diminished hepatic synthetic function (INR 2.8 not responsive to Vitamin K), hyperbilirubinemia (total bilirubin 12.4 mg/dL), and elevated transaminase levels (ALT/AST 1000/705 U/L). Upon stateside evaluation, he developed asterixis and progressive encephalopathy. The history obtained from the patient was reliably negative for toxic ingestions or suspect environmental exposures. The workup for vascular causes, viral and autoimmune hepatitis, Wilson disease, hemochromatosis, and alpha-1-antitrypsin deficiency was negative. An empiric treatment with n-acetylcysteine was ineffective. Per the ALF Study Group estimation, the patient had a 9% 21-day transplant free survival, and was promptly transferred to a transplant center. A liver biopsy was deferred given coagulopathy and rapid decompensation that required vasopressors and intracranial pressure reduction as a bridge to transplant. His explant demonstrated T-cell rich lymphoma with no hyper-metabolic lesions on PET scan. The patient fully recovered from surgery, completed chemotherapy, and remains in clinical remission on his immunosuppressive regimen. DISCUSSION: Less than 40 cases of ALF from hepatic lymphoma have been reported in the literature, with a majority diagnosed post-mortem. Prognosis is generally poor, and rapid clinical deterioration prompts difficult and swift decisions often based on an incomplete data set. As described with this young patient with ALF of initially unknown etiology, proceeding with liver transplant was the only life-saving option. This case demonstrates that a consideration must be made to transplant potentially disqualifying conditions that have effective established therapies.Figure 1.: Diagnostic CD3 stain for T-cell rich lymphoma.Figure 2.: Diagnostic CD20 stain for T-cell rich lymphoma.