Von Willebrand Disease – Board-Style Questions

What this deck covers

Von Willebrand Disease 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. Is this history normal
2. Two methods on offer
3. Normal on a bad day
4. Two numbers just under fifty
5. Activity far below antigen
6. Labelled chronic ITP
7. A haemophilia pattern in a girl
8. Two healthy carriers
9. The brother and the sister
10. First, prove it works
11. One molar to come out
12. After the dose, at home
13. Too young for it
14. Wrong drug for this type
15. A poor responder for theatre
16. Less each time
17. Tranexamic acid was not enough
18. Tired and behind at school

Diagnoses and management options tested

Across the 18 cases you are asked to choose between options such as: A bleeding time performed in the clinic today, Either, since the two always give identical results, Type 3 disease inherited from two unaffected parents, A bone marrow aspirate to examine her megakaryocytes, An assay of how well her VWF binds to factor VIII, Severe haemophilia A, with an X-linked inheritance, Testing the brother only, as boys are affected more, A dose of factor concentrate to compare against it, No cover, since her levels are only mildly reduced, Stop all drinks until the following morning, It does not raise the factor levels in boys at all, It releases no factor at all in type 2B disease, Repeated desmopressin doses twice a day for a week, Stores are depleted by the repeated daily doses.

Clinical pearls from this deck

• Score the story before you send the samples.
• Measure the activity by binding, not by ristocetin.
• A frightened, febrile child can hide the disorder: test again when calm.
• Group O pulls the numbers down; the bleeding history decides the label.
• Ratio below 0.7: look at the multimers.
• Low platelets, a dominant family and low-dose ristocetin: think 2B, not ITP.

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.