FRCPath Haem Part 1 MCQs-Transfusion 483

A 24-year-old man with HbSS is admitted with a severe vaso-occlusive episode and acute chest syndrome. His baseline haemoglobin is 82 g/L. He receives 2 units of appropriately phenotype-matched red cells, with an initial post-transfusion haemoglobin of 81 g/L. He is discharged after clinical improvement.

Nine days later, he is readmitted with severe limb and back pain, fever and dark-coloured urine. His haemoglobin is now 58 g/L. Further investigations show:

• Hb 58 g/L

• Reticulocytes 1.2%  

• Bilirubin increased

• DAT negative

• No new clinically significant red-cell alloantibody detected initially

  
  

He is treated promptly with IVIg 1 g/kg and intravenous methylprednisolone 1 g daily. Twenty-four hours later, his haemoglobin has fallen further to 49 g/L, haemolysis remains marked and his renal function continues to deteriorate.

Which of the following is the most appropriate next treatment?

A. Transfuse 2 units of antigen-matched red cells

B. Rituximab

C. Eculizumab

D. Exchange transfusion  

E. Increase the dose of hydroxycarbamide                                                           F. Tocilizumab