Evaluation of survival of sickle cell patients after liver transplantation at the Henri Mondor Hospital in Créteil (HHM) in France: A Retrospective cohort study

Abstract

Background: Hepatic cholestasis is very common in sickle cell disease, thus requiring liver transplantation; but this technique is not practiced in the developing world. The objective of this study was to evaluate the survival of sickle cell patients after liver transplantation followed at the Henri Mondor Hospital in Créteil (HHM) in France. Methods: Historical cohort study conducted in 24 sickle cell patients followed at the Henri Mondor hospital in Créteil in France during the period from 1991 to 2019. The clinical, biological and evolutionary parameters were studied. Patient survival was described by Kaplan Meier curves and risk factors for death were sought by Cox regression. Results: Among the 24 sickle cell patients who underwent a liver transplant, 11 had died, representing a mortality rate of 45.8%. Their average age was 35.7±8.6 years, female sex ratio 1M/2F. Malnutrition accounted for 25%, 58.3% of patients were homozygous, in hepatic presentation, 45.8% were grade 0 and 54.2% grade I-V. Ascites, hepatic encephalopathy and high risk according to the MIELD score had influenced death (p<0.05). The median time to intervention was longer in the deceased (p<0.001), on the other hand, the duration of follow-up was shorter in the deceased (p<0.001). After adjustment, male gender (HRa: 3.95 95% CI: 1.42-9.00), homozygous status (HRa: 3.92 95% CI: 1.61-5.12), encephalopathy 2.70 (1.41-7.01), MELD score high risk (3.20 (1.26-5.66) and time to intervention ≥ 3 days (HRa: 2.96 95% CI: 1.89-6.78) were the independent predictors of sickle cell mortality. Conclusion: the death rate is high in transplanted sickle cell patients; it is influenced by the state of the liver, the time to intervention and the homozygote state.