Primary Hepatic Amyloidosis Related Liver Failure Rescued with Living Donor Liver Transplant: A Case Report

Background and Aim: Amyloidosis is a systemic infiltrative disorder characterized by deposition of amyloid fibrils in extracellular spaces. Liver involvement can be seen both primary secondly amyloidosis which subclinical or symptomatic. However, hepatic very rare variant protein primarily occurs liver only. We are presenting an interesting case sub-acute failure due to who underwent living donor transplantation (LDLT). Case report: This was 62-year-old female presented with history slowly progressive painless jaundice pedal edema for 4 months abdominal distention 2 weeks. There no past any disease other chronic illness. On history, physical examination initial laboratory evaluation, etiology could not found out. She trans-Juglar biopsy suggestive amyloidosis. initially given steroids, cyclophosphamide bortezomib followed daratumumab lenalidomide non-response therapy. she did respond medical treatment. Due progressively rising bilirubin associated decompensation form ascites encephalopathy, LDLT after thorough evaluation. Her post-operative course smooth uneventful. Serum biochemistry parameters gradually improved. Patient discharged on 18th postoperative day. as successfully LDLT. Primary extremely poor prognosis. Median survival less than nine has been reported patients presence portal hypertension. Conclusion: report describes successful