Beta-thalassaemia major patient on regular transfusions presents with bronze skin, elevated ferritin (4200 ng/mL), and cardiac T2* on MRI of 9 ms (normal >20 ms). Which is the MOST appropriate chelation therapy?
- A Deferasirox alone
- B Combination chelation: deferoxamine + deferiprone ✓
- C Deferoxamine subcutaneous infusion 8–12 hours/night alone
- D Phlebotomy programme
Explanation
Cardiac T2* <10 ms on MRI indicates severe myocardial iron loading, associated with high risk of cardiac failure and arrhythmia. The UK Thalassaemia Society guidelines recommend combination chelation therapy with deferoxamine (continuous infusion or subcutaneous) plus deferiprone for patients with significant cardiac iron loading (T2* <10 ms). Deferiprone has superior cardiac iron clearance due to its low molecular weight and lipophilicity allowing myocardial penetration. Deferasirox alone has less cardiac iron data at this severity. Phlebotomy is contraindicated in transfusion-dependent thalassaemia.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.