SUCCESSFUL LIVER TRANSPLANTATION FOR HEPATO-BILIARY INVOLVEMENT ASSOCIATED WITH SICKLE CELL DISEASE

G Tessier, D Fenyves, D Soulières, AM Nutini, G Ghattas, J Côté, B Nguyen, G Pomier-Layrargues

CHUM, Montréal, Québec

Hepatobiliary involvement in patients with sickle cell disease includes acute sickle hepatic crisis, hepatic sequestrations crisis, sickle cell intrahepatic cholestasis, intra and extra-hepatic cholelithiasis, hepatic iron overload or chronic B or C hepatitis. We report the case of a 20 year old female with long standing sickle cell anemia who was admitted for recurrent cholangitis due to intra and extrahepatic cholelithiasis. Infection could not be controlled despite endoscopic drainage and prolonged antibiotic therapy. This patient was cachectic and her Pugh score was 9/15. She was listed for transplantation which was performed 6 weeks later. Perioperative management include oxygenation, hydratation and blood transfusions to minimize the risk of sick cell crisis. Secondary biliary cirrhosis with intra and extra-hepatic stones, microabscesses, iron overload and sickle cells within the sinusoids were observed in the explanted liver. The patients was maintained on a regimen of tacrolimus, azathioprine and prednisone. Post-operative evolution was uneventful except for a biopsy-proven acute episode of rejection on day 36, treated with pulses of methylprednisolone. On day 46, the patient was readmitted for worsening cholestasis. HBs level was 16%. A liver biopsy demonstrated centrilobular necrosis and numerous sickle cells within the sinusoids; portal inflammation was minimal. A treatment with oxygen, hydratation and intravenous heparin was initiated and the patient was started on an exchange transfusion program. Rapid improvement in liver function tests was noticed and the patient was discharged on day 68. Hepatic sickling recurred 2 weeks later. HBs level was 22%. Exchange transfusions were then performed every 4-6 weeks in order to maintain HBs level below 20%. Liver tests are still normal 8 months after transplantation. This case demonstrates that liver transplantation can be life-saving in this situation but that careful perioperative and post-operative management is mandatory, using a multidisciplinary approach to avoid recurrent episodes of acute hepatic sickling.