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The liver in sickle cell disease.

Christophe Duvoux1, Lorraine Blaise2, Jean-Jacques Matimbo3

  • 1Department of Hepatology and Medical Liver Transplant Unit, Henri Mondor Hospital-APHP, University Paris Est Créteil, France.

Presse Medicale (Paris, France : 1983)
|November 19, 2023
PubMed
Summary

Liver involvement in sickle cell disease (SCD) is common and often missed. Early recognition and multidisciplinary management of SCD hepatopathy are crucial to prevent severe liver damage and improve patient survival.

Keywords:
Auto-immune liver diseasesCholangiopathyLiverLiver transplantationPorto-sinusoidal diseaseSCD hepatopathySickle cell diseaseSinusoidal obstruction syndromeStem cells transplantation

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Area of Science:

  • Hepatology
  • Hematology
  • Vascular Biology

Background:

  • Liver involvement is frequent in sickle cell disease (SCD) but often underestimated, particularly in early or chronic stages.
  • Poorly recognized chronic SCD-related vascular injury can silently progress to end-stage liver disease or acute-on-chronic liver failure (ACLF).
  • Persisting abnormalities in liver function tests (LFTs) in SCD patients warrant thorough investigation.

Purpose of the Study:

  • To emphasize the importance of early recognition and multidisciplinary management of SCD-related liver disease (hepatopathy).
  • To propose an algorithmic approach for investigating persistent LFT abnormalities in SCD patients.
  • To highlight therapeutic options for both early and advanced SCD hepatopathy, including liver transplantation.

Main Methods:

  • Review and synthesis of current knowledge on SCD liver involvement.
  • Proposal of a multidisciplinary diagnostic and management algorithm for SCD hepatopathy.
  • Discussion of therapeutic strategies, including medical management, hematopoietic stem cell transplantation (HSCT), and liver transplantation.

Main Results:

  • Early SCD hepatopathy necessitates revision of SCD management, including potential initiation of transfusion exchanges and hydroxyurea, and control of cofactors.
  • Hematopoietic stem cell transplantation (HSCT) role in early hepatopathy requires further evaluation.
  • Liver transplantation is the only option for improved survival in advanced SCD hepatopathy, with elective transplantation yielding better outcomes than emergent procedures for acute liver failure (ALF) or ACLF.

Conclusions:

  • Multidisciplinary workup and management involving hepatologists are essential for SCD liver disease.
  • Early intervention in SCD hepatopathy can prevent further liver injury and decompensation.
  • Liver transplantation is a critical therapeutic option for advanced SCD hepatopathy, offering improved survival.
  • Establishment of national/international registries and observational cohorts is encouraged to enhance understanding and management of SCD liver diseases.