Chronic Liver Disease in Transfusion-Dependent Thalassemia: Liver Iron Quantitation and Distribution

Abstract

The quantitative and/or qualitative distribution of liver iron was assessed in 81 transfusion-dependent thalassemia major patients with chronic liver disease (36 with chronic active hepatitis, 23 with chronic persistent hepatitis, 22 with siderosis). Viral marker studies showed only 3 cases with both HBsAg and anti-HBc posiüvity in the serum, while the others had anti-HBc and anti-HBs or only anti-HBs or no B viral markers. A significantly higher iron overload was found in chronic hepatitis, particularly chronic active hepatitis, than in siderosis. This increased iron overload may be due to less intensive chelation treatment, higher intestinal absorption secondary to lower mean Hb levels, and/or to liver inflammation-depedent iron deposition. The liver iron overload in turn may facilitate the development or persistence of chronic progressive liver disease.

Benefits and Complications of Regular Blood Transfusion in Patients with Beta-Thalassaemia major

“…In 1984, Jean et al [61]found that substantial liver fibrosis could occur during the first decade of life in thalassaemic children receiving inadequate chelating therapy, and that most children had histologically proven liver cirrhosis in adolescence. In the studies of De Virgiliis et al [62]and Aldouri et al [63], hepatic iron was related to the degree of necroinflammatory activity. It is remarkable, however, that only few studies investigated the role of ‘pure’ iron overload in liver disease of patients with β-TM, since most of the information derives from trials conducted before the discovery of HCV.…”

Relationship between hepatocellular injury and transfusional iron overload prior to and during iron chelation with desferrioxamine: a study in adult patients with acquired anemias

“…Moreover, many of these studies were carried out before the discovery of HCV. [3][4][5] HCV infection is an uncommon cause of liver dysfunction in multiply transfused patients in Denmark where HCV prevalence is low. 6 This afforded us a unique opportunity to study the relationship between transfusional iron overload and liver disease without coexisting liver disease caused by hepatotropic virus infection.…”

Iron Chelation in Transfusion-Dependent Thalassemia with Chronic Hepatitis

“…In a previous study by our group [4] these two factors were found to be related, as higher liver iron overload was seen in thal major patients with chronic hepatitis than in patients with no hepatitis [4]. This suggested that an aggressive iron chelation treatment should be given to transfusiondependent thal major patients with chronic liver disease in order to arrest or slow down the evolution to cirrhosis.…”