Liver transplantation for sickle cell disease: a systematic review

Abstract

Background: 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.

Methods: This study aims to make a systematic review of the current literature to evaluate indications, timing, and results of liver transplantation for patients affected by SCD.

Results: Twenty-nine patients in total were reported worldwide until 2018, the average patient age is 28.7 (0.42-56), all patients have a pre-transplant diagnosis of SCD. Cirrhosis at transplantation was present in six-teen (n = 16, 55.1%) patients. In ten patients (n = 10, 34.5%), acute liver failure arises from healthy liver and presented sickle cell intrahepatic cholestasis. Eleven patients (n = 11, 39.2%) died, three (n = 3, 10.7%) in the first postoperative month, and seven (n = 7, 25%) in the first year. Mean follow-up was 27 months (range: 7-96), one-year overall survival was 48.7%.

Discussion: Liver transplantation for SCD has been increasingly reported with encouraging results. Indications are presently reserved for acute liver failure arising both in healthy liver and end-stage liver disease.