Risks and outcomes of liver transplantation in patients with sickle cell disease

Position du problème et objectif(s) de l’étude

Sickle cell disease is a group of autosomal recessive disorders characterised by haemolytic anaemia. Liver is one of the most affected organs, ranging from liver tests alterations to acute liver failure for which liver transplantation is the only life-saving treatment. Liver transplantation (LT) then becomes a possible treatment. However, data regarding LT in patients with SCD are limited to two small case series. This case series reviewed 13 patients who underwent a LT at hospital since 2019. We aimed to describe the characteristics of the patients at the time of their access to LT and their perioperative outcome.

Matériel et méthodes

This single centre retrospective cohort study analysed 13 consecutive patients who underwent a LT since 2019. Results are expressed as number, percentage, and mean with standard deviation

Résultats & Discussion

Patients had 36 ± 14 years old, (163 ±19 cm and 51± 8 kg). Mean MELD score just before LT was 28 ± 12. Preoperative haemoglobin was 8.0 ± 1.2 g/dl. The main reason of their LT was acute liver failure on cirrhosis (N=7), fulminant hepatitis (N= 2) and autoimmune hepatitis (N=4). All patients were hospitalized before their surgery. One patient was treated before surgery with MARS, and was under assisted mechanical ventilation and norepinephrine infusion. Surgical duration was 530 ± 55 minutes. Intraoperative transfusion was necessary in 12 out of the 13 patients with an average of 3 ± 2 units of packed red blood cells. Five patients needed fresh frozen plasma and 3 patients platelet transfusion. Intraoperative blood loss was 2595 ± 2560 ml. Fluid balance at the end of the surgery was + 2380 ml in median. Lactate concentration at the end of the surgery was 6.0 ± 4.3 mmol/l. Maximum dose of norepinephrine during surgery was on average at 3.2 ± 0.2 mg/h. All patients (except one) were extubated on postoperative day 1. Intensive care unit and hospital length of stays were 9 ± 5 and 40 ± 65 days respectively. All patients presented at least one major postoperative complications within 30 days post-surgery (severe AKI requiring renal replacement therapy (N=4), AKI not requiring RRT (N=7), biliary complication (N=3), portal thrombosis (N=3), retro-hepatic haemorrhage (N= 4), early allograft dysfunction (N=1), subarachnoid haemorrhage (N=2), sepsis (N=3), redo surgery (N=6). The 2 patients who developed subarachnoid haemorrhage died within 30 days post-surgery. A last patient deceased at 1 year after surgery from multiple causes (thrombosis, biliary and infection complications)

Conclusion

LT for severe liver failure in sickle cell patients is feasible but is associated with high morbidity (100%) and mortality rates (15%) during the early postoperative course.

Auteurs

L. Toubal (1), L. Joanna (1), E.C. Cynthia (1), N. Salima (1), A.J.O.O.S. Alexandre (2)

Orateur(s)

Leila TOUBAL (Villejuif)