Background: Transfused thalassaemia patients are at risk of cirrhosis. Transient Elastography (TE) enables non-invasive liver stiffness measurement (LSM). The Enhanced Liver Fibrosis (ELF) score has excellent correlations with liver fibrosis. We aimed to evaluate the prevalence of and risk factors for fibrosis in a cohort of adult patients with transfusion dependent thalassaemia using LSM and ELF testing.
Methods: All adults with transfusion-dependent thalassaemia at our institution were invited to undergo LSM and ELF measurements. Concurrent and historical ferritin, hepatitis C viral load, liver ultrasound and T2* MRI data were obtained.
Findings: We evaluated 63 patients (mean age 43y, 46% male, 89.9% using deferasirox) for liver fibrosis by LSM and ELF. By LSM, 29% had fibrosis, including 11% with cirrhosis. By ELF score, 34.4% had severe fibrosis. By multiple linear regression, LSM was associated with age (P=0.002), presence of detectable hepatitis C virus (HCV) (P=0.002), and both current (P<0.012) and historical ferritin level (P=0.012) concentrations (R2=0.457). By multiple regression, ELF score was associated with age (P=0.005) and historical ferritin levels (P<0.001).
Conclusions: Hepatic fibrosis and cirrhosis are prevalent in adults with transfusion dependent thalassaemia. Historical iron overload and HCV infection mediate fibrosis risk. Most adult patients with a history of prior iron loading and/or untreated HCV should undergo screening for cirrhosis, irrespective of ferritin level.
Edward Shelton, Chia Pei Chong, Lani Shochet, Jamie heong, Sim Yee Ong, Donald K Bowden, Alexander Hodge, Virginia Knight, Ken Cheng, Sant-Rayn Pasricha and Anouk Dev