FRCPath Haem Part 1 MCQs-Transfusion 487

A 36-year-old woman with HbSC disease is being assessed before an elective major abdominal surgery for a large intra-abdominal tumour. She has not previously required regular transfusion and has never been on a chronic transfusion programme. Her baseline haemoglobin is 102 g/L, with a reticulocyte count of 165 × 10⁹/L and bilirubin of 42 µmol/L. She has a history of two previous vaso-occlusive episodes, the most recent 18 months ago, but no previous acute chest syndrome or stroke.

She is currently clinically well with normal oxygen saturations on room air. Pre-operative investigations show:

• Hb 102 g/L

• Platelets 286 × 10⁹/L

• Reticulocytes 165 × 10⁹/L

• HbS 54%

• HbC 42%

• HbF 4%

• Renal and liver function satisfactory

  
  

The anaesthetist asks whether transfusion can be avoided because she has HbSC rather than HbSS disease. Which is the most appropriate peri-operative transfusion strategy?

A. No transfusion because HbSC disease is associated with a milder phenotype

B. Simple transfusion immediately before surgery, aiming to increase the haemoglobin to approximately 120 g/L

C. Simple transfusion only if the haemoglobin falls below 100 g/L peri-operatively

D. Pre-operative exchange transfusion

E. Establish a chronic transfusion programme for 3 months before surgery, aiming for HbS <30%