Morphological study of liver biopsy in Thalassaemia major

Objective: To see the morphological changes in liver in transfusion dependent Thalassaemia major children undergoing bone marrow transplantation. Methods: This retrospective cross-sectional survey was conducted at Pathology department of Army Medical College and Paediatric department of Military Hospital, Rawalpindi from Jul 2000 to Aug 2003. Liver biopsies were done in 40 thalassaemic major children and histological changes including Knodell Histological activity index (HAI); grade, stage and score along with degree of haemosiderosis were noted. Serum ALT levels, ferritin assays and screening for HBsAg and Anti- HCV antibody were also carried out in these cases. Results: Forty children 1.5 - 10.5 years of age (mean 6.1 years) with a male to female ratio of 1.2:1 were included in the study. According to Knodell HAI scoring, 24 (60%) cases had Knodell HAI score between 13/22 to 18/22 and 18 patients (45%) in grade 9-12/18. Six children had fully developed cirrhotic changes whereas 22 and 12 patients showed stage 3 and 1 respectively. Twenty eight (70%) patients had grade 3-4 haemosiderosis. HBsAg was positive in 6 and anti- HCV antibody in 14 patients. Serum ferritin and ALT levels were markedly raised in most of the patients. Conclusion: Seventy percent patients had moderate to severe haemosiderosis and high Knodell HAI score was found in children with severe haemosiderosis, raised ALT and Ferritin levels and with positive serology for HBsAg and anti- HCV antibody. Liver biopsy is useful in thalassaemic children to assess the stage of liver disease and selection of suitable cases for bone marrow transplantation (BMT).