FRCPath Haem Part 1 EMQs-Transfusion 68 and 69

Options

A. No transfusion required

B. Simple transfusion (top up)

C. Exchange transfusion

D. Either simple or exchange transfusion depending on severity

E. Avoid transfusion unless life-threatening anaemia

F. Urgent red cell exchange within hours

G. Chronic transfusion programme

H. Emergency transfusion followed by ICU support

I. Supportive management only

J. Pre-operative transfusion optimisation

K. Automated exchange transfusion

L. Manual exchange transfusion

M. Transfusion contraindicated

N. Immediate neurology-led stroke protocol plus exchange transfusion

O. Observation and repeat haemoglobin assessment

Questions

1.

A 19-year-old woman with HbSS disease presents with worsening fatigue and dizziness following a recent parvovirus infection. Examination shows pallor but no splenomegaly. Hb has fallen from 8.5 g/dL to 3.9 g/dL with a markedly low reticulocyte count. She is haemodynamically stable with no respiratory symptoms.

What is the most appropriate transfusion strategy?

2.

A 7-year-old boy with HbSS disease is brought to hospital with abdominal distension, lethargy, and tachycardia. Examination reveals a rapidly enlarging spleen. Hb is 4.8 g/dL (baseline 8.2 g/dL) with reticulocytosis. Blood pressure is borderline low.

What is the most appropriate transfusion strategy?

3.

A 25-year-old man with sickle cell disease develops severe chest pain, hypoxia, fever, and bilateral pulmonary infiltrates on chest imaging. Oxygen saturation remains 84% despite supplemental oxygen. Hb is close to baseline at 8.0 g/dL.

What is the most appropriate transfusion strategy?

4.

A 30-year-old woman with HbSS disease suddenly develops right-sided weakness and expressive dysphasia. CT head excludes haemorrhage. Symptoms began 2 hours earlier. Hb is 7.9 g/dL.

What is the most appropriate transfusion strategy?

5.

A 33-year-old man with sickle cell disease develops severe sepsis secondary to pneumococcal bacteraemia. He becomes hypotensive and requires ICU admission. Laboratory tests demonstrate rising lactate and worsening hypoxia.

What is the most appropriate transfusion strategy?

6.

A 14-year-old girl with HbSC disease is scheduled for elective laparoscopic cholecystectomy. She has a history of recurrent vaso-occlusive crises but is clinically stable at present.

What is the most appropriate transfusion strategy?

7.

A 27-year-old pregnant woman with HbSS disease presents at 28 weeks gestation with recurrent painful crises requiring multiple admissions. Fetal growth restriction has also been identified on ultrasound.

What is the most appropriate transfusion strategy?

8.

A 22-year-old man with sickle cell disease develops progressive jaundice, tender hepatomegaly, worsening liver function tests, and coagulopathy. Bilirubin is markedly elevated and there is evidence of acute liver failure.

What is the most appropriate transfusion strategy?

9.

A 24-year-old woman with sickle cell disease develops dark urine and severe anaemia 10 days after receiving a blood transfusion. Hb falls to 4.5 g/dL. Antibody testing suggests delayed haemolytic transfusion reaction. She is stable without cardiovascular compromise.

What is the most appropriate transfusion strategy?

10.

A 9-year-old child with HbSS disease undergoes transcranial Doppler screening which demonstrates persistently elevated cerebral blood flow velocities associated with high future stroke risk. The child has never had a stroke.

What is the most appropriate transfusion strategy?