P1453: orthotopic liver transplant in sicilian hemoglobinophaties patients

Topic: 27. Thalassemias Background: Chronic liver failure is one of the most frequent complications in patients (pts) affected by hemoglobinopaties due to multifactorial cause as haemochromatosis, vaso occlusive crisis (VOC) in sickle cell disease (SCD), HCV/HBV infections and carcinoma. Currently no studies investigating the role of orthotopic liver transplant (OLT) in hemoglobinopathic pts with chronic liver failure have been conducted. Aims: The aim of the study is to evaluate the survival time and complications occurred following OLT in hemoglobinopatic pts. Methods: Between 2007 to 2022, 9 cadaveric OLT were performed in hemoglobinopathic pts with chronic liver failure at ISMETT-Palermo. 8 of these pts were followed at the Campus of Hematology Franco and Piera Cutino. Demographic, clinical, laboratory, and transplant data were retrospectively collected and analyzed. Descriptive statistics were presented as median (interquartile range [IQR]), and percentage. Results: Among 9 pts (Tab. I) 6 were thalassemic in periodic transfusion support and 3 affected by SCD; 89.9% had no severe hemochromatosis (thalassemic practiced iron chelation therapy differently to SCD pts). All pts were splenectomized and 44.4% alloimmunized. Infectious cause was found in 88.9% of pts (7 HCV+/1 HBV+) - 75% complicated by hepatocellular carcinoma (HCC) - and 11.1% had VOC injury; hepatic VOC damage was found in all SCD pts. At OLT-time HCV+ pts were all HCV-RNA negative after antiviral treatment: 5 treated with IFN or IFN/ribavirin (3 of them treated afterwards with direct antiviral due to no sustained virological response (SVR) and 2 with direct antiviral therapy obtaining SVR. HBV-DNA+ patient underwent therapy with Entecavir. Among all pts 89.9% is currently alive; only 1 died. None of pts presented intraoperative complications; 77.9% of pts stayed in ICU (Intensive Care Unit) between day +2 and +13 following OLT, only 1 (currently alive) stayed in ICU for 209 days, 1 patient died on day +2 in ICU for primary nonfunction liver. The average post-ICU hospitalization was 30(±39) days and 66.7% presented delayed function lasting 7(±7) days. Infectious and surgical complications were the most common observed. Of all pts 66.7% had bacterial infections during post-OLT hospitalization that decreased to 44.4% at 1-year follow-up. At 6-month follow-up there were 55.6% of infectious complications, 50% from bacteria and 50% from virus (of the latter 2 from CMV and 1 from EBV). 33.3% of pts had surgical complications in the post-OLT hospitalization period, 55.6% at 6 months and 44.4% at 1 year. 11.1% had surgical complication of the biliary tract with bacterial infection during OLT hospitalization, 33.3% at 6-month follow-up and 44.4% at 1-year follow-up. There was hepatic artery stenosis in 11.1% in close post-OLT time, 22.2% at 6 months and in 11.1% at 1 year. No arterial thrombosis was found in any follow up. No portal vein thrombosis was found in the post-OLT hospitalization and at 6-months follow-up; a portal vein thrombosis occurred at 1-year follow-up. Of all pts 11.1% had signs of rejection during post-OLT hospitalization and at 1-year, none at 6 months. Currently, no case of re-occurrence of HCC or other second neoplasm, nor re-OLT were reported. Summary/Conclusion: The study demonstrates that OLT is a valid therapeutic option in hemoglobinopathic pts, despite need of lifelong frequent follow-up and increased complications risk. This stimulates us to evaluate and develop further prevention protocols to reduce infectious risk and surveillance protocols to reduce surgical complications.Keywords: Liver transplant, Hepatitis, Hemoglobinopathy, Liver disease