FRCPath Haem Part 1 MCQs-Gen Haem 441

A 48-year-old man of Irish ancestry is referred to the haematology clinic after abnormal liver function tests are noted during investigation of fatigue. He reports worsening pain in both hands over the past 5 years and states that handshakes have become increasingly uncomfortable. His father died from liver cancer aged 68 years. He drinks less than 10 units of alcohol weekly.

Examination reveals mild skin hyperpigmentation and tenderness over the second and third metacarpophalangeal joints bilaterally..

Test	Result
Ferritin	1180 μg/L
Serum iron	39 μmol/L
Transferrin	1.7 g/L
Transferrin saturation	85%
ALT	115 U/L
Albumin	41 g/L
Bilirubin	Normal

The patient undergoes transient elastography, which demonstrates advanced fibrosis consistent with cirrhosis.

After completion of induction venesection, his ferritin falls to 25 μg/L and transferrin saturation to 42%.

Which ONE of the following statements is MOST CORRECT regarding his ongoing management and prognosis?

A. No further treatment is required

B. Maintenance venesection should continue, targeting ferritin <50 μg/L

C. Maintenance venesection should continue until ferritin becomes undetectable (<10 μg/L)

D. The presence of C282Y homozygosity alone justifies 6-monthly liver ultrasound surveillance irrespective of cirrhosis status.

E. Iron chelation therapy should replace venesection because cirrhosis is present.