First Successful Liver-Alone Transplantation for TERT (Telomerase Reverse Transcriptase)-Telomeropathy-Related Hepatoportal Sclerosis Cirrhosis

Hepatoportal sclerosis is a rare but well-described condition leading to end-stage liver disease. Telomeropathy is a rare genetic disorder which manifests as premature senescence of cells leading to multisystem disease involving bone marrow, lungs and skin. To the best of our knowledge, there is no report of telomeropathy precipitating end-stage liver disease. Our case presented hepatopulmonary syndrome. Herein, we report a successful liver transplantation in a patient who suffered hepatoportal cirrhosis from telomerase reverse transcriptase (TERT)-telomeropathy.