Successful Orthotopic Liver Transplantation in an Adult Patient with Sickle Cell Disease and Review of the Literature

Abstract

Sickle cell disease can lead to hepatic complications ranging from acute hepatic crises to chronic liver disease including intrahepatic cholestasis, and iron overload. Although uncommon, intrahepatic cholestasis may be severe and medical treatment of this complication is often ineffective. We report a case of a 37 year-old male patient with sickle cell anemia, who developed liver failure and underwent successful orthotopic liver transplantation. Both pre and post-operatively, he was maintained on red cell transfusions. He remains stable with improved liver function 42 months post transplant. The role for orthotopic liver transplantation is not well defined in patients with sickle cell disease, and the experience remains limited. Although considerable challenges of post-transplant graft complications remain, orthotopic liver transplantation should be considered as a treatment option for sickle cell disease patients with end-stage liver disease who have progressed despite conventional medical therapy. An extended period of red cell transfusion support may lessen the post-operative complications.

Guidelines on red cell transfusion in sickle cell disease Part II: indications for transfusion

“…In patients with renal disease, transfusion may be considered in end stage renal disease, in those awaiting a renal transplant and post‐renal transplant (Sharpe & Thein, ). Transfusion has also been used in patients undergoing liver transplantation in the peri‐transplant setting (Blinder et al , ; Gardner et al , ). Blood transfusion in the treatment of pulmonary hypertension seems theoretically reasonable, with the aim of decreasing haemolysis and thereby nitric oxide scavenging and consequent pulmonary vasoconstriction, and has been recommended for this purpose (Machado & Gladwin, ; Cho & Hambleton, ).…”

Plasmapheresis in the Treatment of Acute Liver Failure: A Case Report and a Review of the Literature

“…In this case, there was an extreme hyperbilirubinemia and, therefore, liver transplantation was indicated (6). Although there are case reports of successful liver transplantation for sickle-cell disease, the experience is still limited (7). Nevertheless, the patient was not found to be eligible for transplantation by our transplantation team because of very low success rates in this group of patients.…”

Acute liver function decompensation in a patient with sickle cell disease managed with exchange transfusion and endoscopic retrograde cholangiography

“…Delis and colleagues described a 43-year-old patient with SCD for whom ET successfully reduced the HbS level from 35% to 7.3% and reversed the intrahepatic cholestasis that occurred after surgical resection of liver metastases due to colon cancer [Delis et al 2006]. Similarly, in a recent review, Blinder and colleagues measured pre-and post-transplant HbS levels with a pre-transplant HbS goal of <30% and a post-transplant HbS goal of <2% in a patient with SCIC to monitor efficacy of treatment using ET [Blinder et al, 2013].…”