Haemophilia Therapy Standardisation Board. Non-genetic risk factors and the development of inhibitors in haemophilia: a comprehensive review and consensus report.

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Results: Liver stiffness was significantly correlated with thalassemia classification (p 5 0.001), iron deposition (p 5 0.003) and fibrosis stage (p\0.001). Median TE values were significantly different in patients with severe fibrosis (stage 3-5) 4.5 (60.8 SD) kPa from those with mild or no fibrosis (stage 0-2) 3.9 (62.8 SD) kPa (p 5 0.000). The AUROC for prediction of high fibrosis stages (3-5) was 0.802 (95% confidence interval [CI]: 0.665-0.938) with cutoff of 4.35 kPa with 81.8% sensitivity (95% CI: 52.3-94.9) and 70.0% specificity (95% CI: 52.1-83.3). Conclusion: Our results demonstrate that transient elastography is a reliable non-invasive tool to evaluate liver iron content and fibrosis in patients with b-thalassemia major who are candidates for hematopoietic stem cell transplantation and can be an alternative to liver biopsy. Moreover, it is a valuable tool to follow-up the liver status in patients with b-thalassemia major after transplantation.