Hereditary Spherocytosis – Board-Style Questions

What this deck covers

Hereditary Spherocytosis 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. What lies ahead
2. A clean family history
3. Going home on day five
4. Blood every few weeks
5. Mother and grandfather
6. Not quite typical
7. Playing football
8. His sister next
9. A sore throat first
10. Parents want it out now
11. Most but not all
12. The preoperative checklist
13. Four days to go
14. A September question
15. Rigors at fourteen
16. Eight days after surgery
17. Three months later
18. Booking visit

Diagnoses and management options tested

Across the 18 cases you are asked to choose between options such as: Milder, since boys are affected less than girls are, Their normal tests mean her diagnosis must be wrong, An ultrasound of the spleen at six weeks of age, The eosin-5-maleimide binding test by flow cytometry, Mild disease, for, Nothing, since she has no symptoms at present, A transient aplastic crisis from parvovirus B19, Remove the whole spleen now, for immediate benefit, It cures the anaemia fully and needs no vaccinations, A bone marrow aspirate to exclude another cause, No other vaccine, as his course is complete, Thrombosis in the portal venous system, An accessory spleen has taken over and needs removal, Removal of her spleen in the second trimester.

Clinical pearls from this deck

• In dominant spherocytosis, the parent’s course is the best forecast.
• No family history does not mean no hereditary spherocytosis.
• In a newborn with spherocytosis, the jaundice comes first and the anaemia follows.
• Transfused in infancy is not the same as transfusion dependent.
• Typical picture plus family history: the diagnosis is made.
• An atypical ‘spherocytosis’ is confirmed before anyone takes the spleen.

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.