FRCPath Haem Part 1 MCQ-Gen Haem 446

A 29-year-old woman with transfusion-dependent β-thalassaemia major attends her annual comprehensive iron overload review. She has received approximately 180 units of packed red blood cells since childhood and is currently receiving deferasirox 21 mg/kg/day, with excellent reported adherence.

Examination reveals:

• Mild bronze skin pigmentation

• Liver palpable 3 cm below the costal margin

Test	Result
Hb	95 g/L
Ferritin	2,650 µg/L (stable for 18 months)
Transferrin saturation	91%
ALT	58 U/L (NR <40)
LH/FSH	Low
Oestradiol	Low

Echocardiography shows: LVEF 56%

MRI findings: Cardiac T2 = 9 ms*

Liver iron concentration = 15 mg/g dry weight

The multidisciplinary iron overload team decides to intensify treatment.

Which ONE of the following is the strongest justification for escalating to combination chelation therapy rather than simply continuing her current regimen?

A. The persistently elevated ferritin concentration (>2,500 µg/L) despite maximal-dose deferasirox alone

B. The liver iron concentration exceeds the threshold associated with hepatic fibrosis

C. Severe myocardial iron loading demonstrated by cardiac MRI with evidence of early iron-related organ dysfunction despite adequate monotherapy

D. Transferrin saturation above 90%, indicating complete saturation of transferrin

E. The presence of multiple endocrine abnormalities, which are irreversible once established