Management of liver complications in haemoglobinopathies

  • Pierre Brissot | pierre.brissot@univ-rennes1.fr Service des Maladies du Foie, Hôpital Pontchaillou, Rennes University Hospital, Rennes, France.
  • Olivier Loreal Service des Maladies du Foie, Hôpital Pontchaillou, Rennes University Hospital, Rennes, France.

Abstract

Liver complications in haemoglobinopathies (thalassaemia and sickle cell disease) are due to several factors, dominated (beside chronic viral infections, not considered here) by chronic iron overload, biliary obstruction and venous thrombosis. Whereas the latter two factors can cause acute hepatic syndromes, all three mechanisms - when becoming chronic- can produce fibrosis and cirrhosis and even, in thalassaemia, hepatocellular carcinoma. These chronic hepatic complications are an indirect consequence of the significant improvement in life expectancy due to the overall amelioration of disease management. The diagnostic approach has benefited from non invasive (biochemical and imaging) approaches which have considerably reduced the indication of liver biopsy. The therapeutic management involves relatively efficient curative medical, endoscopic or surgical methods, but should rest primarily on preventive measures focused on the haematological causative factors but also on hepatic co-morbidities. This chapter will focus on hepatic complications in thalassaemia and sickle cell disease (SCD), without considering the complications related to virus B or C infections which will be described in another chapter.

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Published
2014-12-04
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Issue
Section
SESSION 3: Multidisciplinary care in haemoglobinopathies and rare anaemias
Keywords:
thalassaemia, sickle cell diseas, hemoglobinopathies, chronic iron overload, hepatocellular carcinoma.
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How to Cite
Brissot, P., & Loreal, O. (2014). Management of liver complications in haemoglobinopathies. Thalassemia Reports, 4(3). https://doi.org/10.4081/thal.2014.4869