• Presse Med · Dec 2023

    The liver in sickle cell disease.

    • Christophe Duvoux, Lorraine Blaise, Jean-Jacques Matimbo, Francky Mubenga, Norbert Ngongang, Monika Hurtova, Alexis Laurent, Jérémy Augustin, Julien Calderaro, Edouard Reizine, Alain Luciani, Anoosha Habibi, Dora Bachir, Geoffroy Vole, Justine Gellen-Dautremer, Vincent Leroy, Eric Levesque, and Pablo Bartolucci.
    • Department of Hepatology and Medical Liver Transplant Unit, Henri Mondor Hospital-APHP, University Paris Est Créteil, France. Electronic address: christophe.duvoux@aphp.fr.
    • Presse Med. 2023 Dec 1; 52 (4): 104212104212.

    AbstractLiver involvement in SCD patients is frequent but often misdiagnosed or underestimated, except in case of advanced liver diseases. Because of so far poorly recognized forms of chronic SCD-related vascular injury that can silently evolved towards end stages or facilitate ACLF, any persisting liver function tests abnormalities should be carefully investigated, following the above proposed algorithm. Work up and management must be considered multidisciplinary in relationship with a Hepatologist. Early SCD hepatopathy should prompt revision of SCD management to prevent further liver injury and decompensation, discussing transfusion exchanges and hydro urea when not yet initiated, and control for any cofactor of liver injury. The role of HSCT in early SCD hepatopathies also deserves evaluation. In advanced SCD hepatopathies, liver transplantation, which has been rarely performed so far, is the only therapeutic option associated with improved survival. It should definitely be discussed- either electively in case of decompensation in SCD cirrhosis or jaundice/recurrent cholangitis in cholestatic diseases, with excellent outcome, - or emergently in case of ALF or ACLF with more mitigate results. To improve knowledge and management of SCD liver diseases, creation of national and international registries, as well as longitudinal observational cohorts are encouraged.Copyright © 2023. Published by Elsevier Masson SAS.

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