Liver transplantation in patients with sickle cell disease: possible but challenging—a cohort study

Abstract

The liver is frequently affected in patients with sickle cell disease (SCD), but few reports have described liver transplantation (LT) in patients with SCD. We present a thorough analysis of the largest single-center series of LT in patients with SCD and the first systematic review. There were 21 patients with a median age of 37.6 years. LT was performed for acute liver failure related to the sickling process (57%) or electively for end-stage liver disease (43%). Prior to LT, 13 patients (62%) were in the intensive care unit and required mechanical ventilation (33%), vasopressor therapy (24%), renal replacement therapy (10%), or molecular adsorbent recirculating system therapy (19%). Post-LT morbidity and mortality were 95% and 33%, respectively. Patient survival at 1 and 5 years were 58.3% and 41.7%, respectively, in the urgent group and 88.9% and 77.8%, respectively, in the elective group. A total of 22 transplant patients with SCD are described in 20 articles in the literature. The 1-and 5-year patient survival rates for the 18 evaluable patients were 75% and 65%, respectively. LT improves survival in patients with SCD and acute liver failure or end-stage liver disease but is associated with high morbidity during the early postoperative course.

Re: Manuscript titled, “Liver transplantation for sickle cell disease: a systematic review”

“…Similar results were recently published from France, with data from 21 patients from their national referral center along with 22 patients identified via systematic review. 3 Our U.S. analysis of 28 patients showed a 1-year survival of 88.5%, with the French study reporting similar results for patients with chronic liver disease. 2,3 This systematic review only included studies through 2018, reporting on 28 patients with 48.7% 1-year survival.…”

Biomarkers of clinical severity in treated and untreated sickle cell disease: a comparison by genotypes of a single center cohort and African Americans in the NHANES study

“…36 SCD causes a variety of pathologies in the liver, but therapy of SCD can additionally cause liver injury from transfusion-related iron overload and viral hepatitis. 37,38 The frequency of cirrhosis at autopsy has been reported to be as high as 11-14%. [38][39][40] Untreated severe SCD patients in this study had higher alanine transaminase (ALT) and ALP than the NHANES controls.…”

“…At the same time, the urinary albumin to creatinine ratio was significantly elevated in severe SCD phenotypes versus NHANES controls, reflecting the predisposition to chronic kidney disease despite the higher eGFR 36 . SCD causes a variety of pathologies in the liver, but therapy of SCD can additionally cause liver injury from transfusion‐related iron overload and viral hepatitis 37,38 . The frequency of cirrhosis at autopsy has been reported to be as high as 11‐14% 38–40 .…”

RE: re: manuscript titled, “Liver transplantation for sickle cell disease: a systematic review”

“…The letters states that we did not consider the US and French studies made in November and October 2020, respectively. 3,4 We would like to highlight that our analysis was finalised in the mid part of 2020, with the last included report on the subject published in 2019, and submitted the manuscript in the same publication period of the two above cited studies. 3,4 Nevertheless, we discussed them during the review phase, including in the discussion the results published by Hogen 3 and Levesque, 4 considering them as a reference of highvolume centres, and supporting the idea that the increasing number of patients that underwent LT for SCD and the most recent reported results encourage to keep going in this direction.…”

“…3,4 We would like to highlight that our analysis was finalised in the mid part of 2020, with the last included report on the subject published in 2019, and submitted the manuscript in the same publication period of the two above cited studies. 3,4 Nevertheless, we discussed them during the review phase, including in the discussion the results published by Hogen 3 and Levesque, 4 considering them as a reference of highvolume centres, and supporting the idea that the increasing number of patients that underwent LT for SCD and the most recent reported results encourage to keep going in this direction. Given the rarity of the indication, and the relatively small number of treated patients in a long period, only a word of caution was added, suggesting that this subgroup of patients should be addressed to high volume LT centers with concomitant experience in the management of SCD.…”