Thalassaemia and Chronic Transfusion in Children — Board-Style Questions

What this deck covers

Thalassaemia and Chronic Transfusion in 18 board-style clinical cases with worked explanations. Each case gives you a clinical vignette, four options, the correct answer, and an explanation of why each of the other three is wrong, with the guideline or textbook it follows named on the answer.

Board-style teaching questions for paediatricians and trainees preparing for paediatric board, MRCPCH and licensing examinations. Written from published guidelines and standard paediatric textbooks, with the source named on every answer.

The 18 cases, in order

1. Pale from the middle of his first year
2. Her mother was told the same for years
3. Chromatography that showed nothing at all
4. Two carrier parents and a swollen fetus
5. Blue dots brought out by a special stain
6. Why treat him while he still feels well
7. Get her typed properly from the start
8. The organ that decides how long he lives
9. Nightly needles the family cannot keep up
10. A fever that must never wait until morning
11. No sign of puberty at fifteen
12. A crushed vertebra at seventeen
13. Bones that widened to keep up
14. The volume keeps climbing every year
15. Fifteen minutes in, and it went wrong
16. His sister turned out to be a perfect match
17. She is only nine weeks pregnant
18. Barely any blood, and heavy with iron

Diagnoses and management options tested

Across the 18 cases you are asked to choose between options such as: Haemoglobin H disease, in, Between fifty and sixty grams per litre, Oral deferasirox once daily, Lifelong transfusion with intensive chelation.

Clinical pearls from this deck

• Nothing changes at six months except fetal haemoglobin. That is the clock this disease runs on.
• Correct the iron first. Deficiency drags the A2 fraction down and hides the carrier.
• A normal A2 does not exclude thalassaemia. It just moves you from the beta genes to the alpha ones.
• Why can beta disease never present before birth? Because fetal haemoglobin does not use beta chains.
• Fine blue inclusions on a supravital stain, with a normal A2, means the alpha genes.
• You are not transfusing the haemoglobin. You are transfusing to switch the marrow off.

Work through the deck once for recognition, then again a week later to test yourself — each question gives the answer and explains why every other option fails.

More from this system: all Haematology case decks · pediatric reference values.