ORIGINAL ARTICLE

Liver disease in transfusion dependent thalassaemia major

C K Li¹, K W Chik¹, C W K Lam², K F To³, S C H Yu⁴, V Lee¹, M M K Shing¹, A Y K Cheung⁵, P M P Yuen¹

¹ Department of Paediatrics, Prince of Wales Hospital, The Chinese University of Hong Kong
² Department of Chemical Pathology, Prince of Wales Hospital
³ Department of Anatomical and Cellular Pathology, Prince of Wales Hospital
⁴ Department of Diagnostic Radiology and Organ Imaging, Prince of Wales Hospital
⁵ Centre for Clinical Trial and Epidemiological Research, Prince of Wales Hospital

Correspondence to:
Correspondence to:
Dr C K Li, Department of Paediatrics, Prince of Wales Hospital, Shatin, Hong Kong SAR, China;
ckli{at}cuhk.edu.hk

Aims: To study the prevalence and severity of liver diseases of transfusion dependent thalassaemia major patients, and correlate the histological and biochemical changes of iron overload in liver with the peripheral blood markers.

Method: Liver biopsy was performed to assess the histological changes and liver iron content (LIC).

Results: One hundred patients were evaluated (median age 11.7 years, range 1.5–27). A total of 81 liver biopsies were performed in 73 patients; 43 samples were analysed for LIC. Grade 3–4 haemosiderosis and hepatic fibrosis was found in 44% and 30% of patients respectively; both were significantly associated with higher serum ferritin, liver enzymes, and LIC. Very high LIC (>15 mg/g dry weight) was present in 16.3% of patients.

Conclusion: Severe haemosiderosis and hepatic fibrosis were common in patients with thalassaemia major despite the use of chelation therapy. Liver biopsy provided information on fibrosis and LIC which could not be accurately predicted from peripheral blood markers.

Keywords: haemosiderosis; liver iron content; thalassaemia

Abbreviations: ALT, alanine aminotransferase; BMT, bone marrow transplantation; LIC, liver iron content; TD, transfusion dependent; TM, thalassaemia major

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