Liver disease in transfusion dependent thalassaemia major.
OBJECTIVE
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.


METHODS
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.


CONCLUSIONS
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.
Li
C K
CK
Department of Paediatrics, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, Hong Kong SAR, China. ckli@cuhk.edu.hk
Chik
K W
KW
Lam
C W K
CW
To
K F
KF
Yu
S C H
SC
Lee
V
V
Shing
M M K
MM
Cheung
A Y K
AY
Yuen
P M P
PM
D000293:Adolescent; D000328:Adult; D000410:Alanine Transaminase / Q000097:blood; D000704:Analysis of Variance; D001706:Biopsy / Q000379:methods; D002648:Child; D002675:Child, Preschool; D003676:Deferoxamine / Q000627:therapeutic use; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use; D008103:Liver Cirrhosis / Q000201:enzymology / Q000209:etiology* / Q000473:pathology; D008297:Male; D012189:Retrospective Studies; D017086:beta-Thalassemia / Q000188:drug therapy / Q000473:pathology*
D007502:Iron Chelating Agents; D005293:Ferritins; D000410:Alanine Transaminase; D003676:Deferoxamine