FRCPath Haem Part 1 MCQs-Gen Haem 471

A 34-year-old Afro-Caribbean woman with HbSS sickle cell disease is admitted with a 24-hour history of fever, right upper quadrant pain and vomiting. Ultrasound confirms acute calculous cholecystitis, and the hepatobiliary surgical team plans an urgent laparoscopic cholecystectomy within the next 24 hours.

Her medical history includes:

• Previous acute chest syndrome (ACS) requiring mechanical ventilation 2 years ago

• Pulmonary hypertension (estimated pulmonary artery systolic pressure 55 mmHg on echocardiography)

• Three vaso-occlusive crises requiring admission during the previous year

• More than 40 lifetime red cell transfusions

She is not taking hydroxycarbamide because of severe neutropenia during previous treatment.

• Hb electrophoresis:

  • HbS 91%

  • HbF 8%

  • HbA 1%

The blood bank reports that compatible donor units are difficult to source because of her alloantibodies.

Which ONE of the following is the most appropriate overall transfusion strategy?

A. Proceed directly to surgery without transfusion because laparoscopic surgery is considered low risk.

B. Give two units of ABO-compatible packed red cells to increase the haemoglobin to approximately 120 g/L before surgery.

C. Perform urgent automated red cell exchange using HbS-negative, leucocyte-depleted, Rh- and extended phenotype-matched, K-negative, Jkᵃ-negative and Fyᵃ-negative red cells, aiming for HbS <30%, while avoiding a post-exchange haemoglobin >110 g/L.

D. Delay surgery until hydroxycarbamide has increased HbF to >20%, then perform simple transfusion.

E. Give prophylactic fresh frozen plasma together with one unit of irradiated red cells to minimise peri-operative complications.