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FRCPath Haem Part 1 MCQs-Gen Haematology 465

A 29-year-old Pakistani woman with β-thalassaemia intermedia is reviewed in the haemoglobinopathy clinic. She has never required a regular transfusion programme but has received occasional red cell transfusions during pregnancy and severe infections.


Over the past year, she reports worsening fatigue and reduced exercise tolerance. On examination, she has frontal bossing, hepatomegaly (4 cm), and splenomegaly (7 cm).


Her investigations show:

  • Hb: 73 g/L

  • MCV: 57 fL

  • Reticulocyte count: Elevated

  • Ferritin: 2,400 µg/L

  • Transferrin saturation: 68%

  • Liver MRI: Moderate iron overload

  • Cardiac MRI T2*: Normal

  • Echocardiogram: Normal pulmonary artery pressure


What is the most appropriate next step in management?


A. Start deferasirox because iron overload is the only indication for treatment.

B. Commence a regular transfusion programme to improve symptomatic anaemia and suppress ineffective erythropoiesis.

C. Continue observation because her Hb is above 70 g/L.

D. Perform splenectomy because splenomegaly is present.

E. Prescribe oral iron supplementation to improve haemoglobin


 
 
 

1 Comment

adwaanhemay
12 hours ago

Answer: B. Commence a regular transfusion programme.


Explanation

This patient has non-transfusion-dependent β-thalassaemia (thalassaemia intermedia) but has now developed clear clinical indications for regular transfusion:

  • Symptomatic anaemia (fatigue, reduced exercise tolerance)

  • Progressive skeletal changes (frontal bossing), indicating ongoing ineffective erythropoiesis

  • Significant hepatosplenomegaly


Although her ferritin is markedly elevated, iron overload is treated with iron chelation, not by withholding or initiating transfusion. The decision to start regular transfusions is based on clinical symptoms and complications, not on haemoglobin level or ferritin alone.


Why the other options are incorrect


  • A. Incorrect – Iron chelation is required for iron overload but does not address symptomatic anaemia or marrow expansion.


  • C. Incorrect – There is no fixed Hb threshold for transfusion in β-thalassaemia intermedia.…


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